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Kasper P, Fischer AK, Schumacher JC. Hepatic sinusoidal obstruction syndrome after immune-checkpoint-inhibitor therapy: when the liver sends SOS. BMJ Case Rep 2024; 17:e260066. [PMID: 39349302 DOI: 10.1136/bcr-2024-260066] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/02/2024] Open
Abstract
A woman in her 50s presented to the hospital with new-onset jaundice and ascites. Her medical history included a melanoma of the right shoulder, diagnosed 8 months before, for which she had received four cycles of ipilimumab and nivolumab. Due to the oncological history and previous immunotherapy, an immune-checkpoint-inhibitor (ICI)-induced hepatitis was suspected, and a high-dose corticosteroid treatment was initiated. However, as there was no improvement in the liver function tests, a percutaneous liver biopsy was performed for further diagnostic clarification. Histological analysis ultimately identified a hepatic sinusoidal obstruction syndrome (SOS) as the underlying cause of liver dysfunction and portal hypertension. ICI-related hepatic SOS represents an extremely rare adverse event related to immunotherapy that should be considered in all patients with treatment-refractory ICI-related hepatotoxicity and clinical signs of portal hypertension.
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Affiliation(s)
- Philipp Kasper
- Department of Gastroenterology and Hepatology, University Hospital Cologne, Cologne, North Rhine-Westphalia, Germany
| | - Anne Kristin Fischer
- Department of Pathology, University Hospital Cologne, Cologne, North Rhine-Westphalia, Germany
| | - Jan Christoph Schumacher
- Department of Gastroenterology and Hepatology, University Hospital Cologne, Cologne, North Rhine-Westphalia, Germany
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Gómez-Centurión I, Gallardo Morillo AI, Pérez Martínez A, Cabrero Calvo M, Chinea A, González L, Pedraza A, Jiménez Lorenzo MJ, Robles MC, Bailén R, Cascón MJP, Cabero A, Piñana Sánchez JL, Luna A, Perera Alvarez M, Rovira M, Torrent Catarineu A, Sánchez-Pina J, Kwon M. Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease after Unmanipulated Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplantation Cyclophosphamide: A Study on Behalf of the Spanish Hematopoietic Stem Cell Transplantation and Cellular Therapy Group (GETH). Transplant Cell Ther 2024; 30:914.e1-914.e8. [PMID: 38851323 DOI: 10.1016/j.jtct.2024.06.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Revised: 05/13/2024] [Accepted: 06/03/2024] [Indexed: 06/10/2024]
Abstract
Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication after both autologous and allogeneic hematopoietic stem cell transplantation (HSCT). However, its characterization after haploidentical HSCT (haplo-HSCT) with post-transplantation cyclophosphamide (PT-Cy) is scarce. This study aimed to describe characteristics and outcomes of patients with SOS/VOD after haplo-HSCT with PT-Cy. We conducted a retrospective study of 797 patients undergoing a haplo-HSCT with PT-Cy between 2007 and 2019 in 9 centers in Spain. SOS/VOD was defined according to modified Seattle, Baltimore, or revised European Society for Blood and Marrow Transplantation (EBMT) criteria. Severity was graded retrospectively according to revised EBMT severity criteria into 4 categories: mild, moderate, severe, and very severe. From a total of 797 haplo-HSCTs performed, 46 patients (5.77%) were diagnosed with SOS/VOD at a median of 19 days (range, 4 to 84 days) after transplantation. Based on revised EBMT severity criteria, the SOS/VOD cases were classified as mild (n = 4; 8.7%), moderate (n = 10; 21.7%), severe (n = 12; 26.1%), and very severe (n = 20; 43.5%). Overall, 30 patients (65%) achieved SOS/VOD complete response, 25 (83%) of whom were treated with defibrotide. Twenty patients (43%) died before day +100 post-HSCT. Death was attributed to SOS/VOD in 11 patients, and 5 patients died of other causes without resolution of SOS/VOD. The incidence of SOS/VOD after haplo-HSCT with PT-Cy was comparable to those reported after HLA-identical HSCT series. Most of the patients developed very severe SOS/VOD according to revised EBMT severity criteria. Despite a promising SOS/VOD complete response (CR) rate (65%), 100-day mortality remained high (43%), indicating that further improvement in the management of this potentially fatal complication is needed.
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Affiliation(s)
- Ignacio Gómez-Centurión
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Hematology, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain.
| | | | | | - Mónica Cabrero Calvo
- Department of Hematology, Hospital Universitario de Salamanca, Salamanca, Spain; Department of Hematology, Instituto de Investigación Biomédica de Salamanca, Salamanca, Spain
| | - Anabelle Chinea
- Department of Hematology, Hospital Regional Universitario de Málaga, Málaga, Spain; Department of Hematology, Hospital Ramón y Cajal, Madrid, Spain
| | - Leslie González
- Department of Hematology, Hospital Regional Universitario de Málaga, Málaga, Spain; Department of Hematology, Hospital Universitario de Gran Canaria Doctor Negrín, Gran Canaria, Spain
| | - Alexandra Pedraza
- Department of Hematology, Hospital Regional Universitario de Málaga, Málaga, Spain; Department of Hematology, Hospital Clinic, Barcelona, Spain
| | - María Josefa Jiménez Lorenzo
- Department of Hematology, Hospital Regional Universitario de Málaga, Málaga, Spain; Department of Hematology, Institut Català d'Oncologia-Hospital Universitari Germans Trias i Pujol, Badalona, Spain
| | - María Calbacho Robles
- Department of Hematology, Hospital Regional Universitario de Málaga, Málaga, Spain; Department of Hematology, Hospital 12 de Octubre, Madrid, Spain
| | - Rebeca Bailén
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Hematology, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain
| | | | - Almudena Cabero
- Department of Hematology, Hospital Universitario de Salamanca, Salamanca, Spain; Department of Hematology, Instituto de Investigación Biomédica de Salamanca, Salamanca, Spain
| | | | - Alejandro Luna
- Department of Hematology, Hospital Ramón y Cajal, Madrid, Spain
| | - Mar Perera Alvarez
- Department of Hematology, Hospital Universitario de Gran Canaria Doctor Negrín, Gran Canaria, Spain
| | | | - Anna Torrent Catarineu
- Department of Hematology, Institut Català d'Oncologia-Hospital Universitari Germans Trias i Pujol, Badalona, Spain
| | | | - Mi Kwon
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Hematology, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain.
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Thielemans N, De Beule N, Van den Bergh F, Lefesvre P, De Becker A. Successful Treatment of Very Severe Sinusoidal Obstruction Syndrome After Gemtuzumab Ozogamicin With Transjugular Intrahepatic Portosystemic Shunt, Defibrotide, and High-Dose Corticosteroids: A Case Report. Cureus 2024; 16:e67682. [PMID: 39185292 PMCID: PMC11344603 DOI: 10.7759/cureus.67682] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/24/2024] [Indexed: 08/27/2024] Open
Abstract
Sinusoidal obstruction syndrome (SOS) is a rare but potentially life-threatening complication, usually described in the setting of hematopoietic stem cell transplantation (HSCT). The very severe forms have a high mortality rate (>80%) and need fast recognition and urgent treatment. In this case report, we describe a unique and successful treatment strategy. We present a 27-year-old patient with newly diagnosed CD33+ acute myeloid leukemia (AML). She was treated with induction chemotherapy (7+3 regimen) and gemtuzumab ozogamicin (GO). In the absence of other major risk factors, she developed a very severe SOS with multiple organ failure. She was successfully treated with the urgent insertion of a transjugular intrahepatic portosystemic shunt (TIPS), defibrotide, and high-dose corticosteroids. This case of successful treatment for very severe SOS supports a combination strategy involving the immediate mechanical reduction of portal hypertension through TIPS and drug-mediated inhibition of microvascular thrombosis. Furthermore, this case shows the need for an improved prevention strategy, including the identification of additional risk factors and biomarkers.
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Affiliation(s)
- Naomi Thielemans
- Department of Clinical Hematology, Universitair Ziekenhuis Brussel, Brussels, BEL
| | - Nathan De Beule
- Department of Clinical Hematology, Universitair Ziekenhuis Brussel, Brussels, BEL
| | - Frans Van den Bergh
- Department of Interventional Radiology, Universitair Ziekenhuis Brussel, Brussels, BEL
| | - Pierre Lefesvre
- Department of Anatomopathology, Universitair Ziekenhuis Brussel, Brussels, BEL
| | - Ann De Becker
- Department of Clinical Hematology, Universitair Ziekenhuis Brussel, Brussels, BEL
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Rabiee A, Cornman-Homonoff J, Kunstman JW, Garcia-Tsao G, Taddei TH. Interventional Radiology and Surgical Treatment Options for Non-Cirrhotic Portal Hypertension. CURRENT HEPATOLOGY REPORTS 2023; 22:269-275. [DOI: 10.1007/s11901-023-00617-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 09/08/2023] [Indexed: 01/04/2025]
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Ramic L, Speckert M, Ramphal R, Ling SC, Temple M, Kehar M. Successful Transjugular Portosystemic Shunt Treatment of Pediatric Sinusoidal Obstruction: Case Report and Review of Literature. JPGN REPORTS 2023; 4:e355. [PMID: 38034423 PMCID: PMC10684189 DOI: 10.1097/pg9.0000000000000355] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/26/2023] [Accepted: 07/26/2023] [Indexed: 12/02/2023]
Abstract
Background In adults with medically refractory sinusoidal obstruction syndrome (SOS), a transjugular intrahepatic portosystemic shunt (TIPS) has been used successfully to improve portal hypertension and symptoms such as ascites. There is limited data on the use of TIPS for SOS in pediatric patients. Methods The index case was reviewed retrospectively. PubMed and Medline databases were searched to identify other cases. Results A 4-year-old male with high-risk neuroblastoma, developed SOS after tandem autologous stem cell transplant. He was medically managed with defibrotide, diuretics, and peritoneal drainage, but, due to refractoriness, he underwent TIPS day +54 following bone marrow transplant. Hepatic venous pressure gradient improved from 17 to 8 mm Hg following TIPS placement with significant improvement in the patient's clinical status and ascites. However, 15 months later, his shunt remained patent, and he remains clinically well with stable liver enzymes. A literature review identified 13 pediatric cases of TIPS for SOS due to varied causes. TIPS caused a median hepatic venous pressure gradient of 9 mmHg (range, 2-38 mm Hg). The mortality following the procedure was 15%, with 2 cases who died at 2- and 11-days post-TIPS. At the time of the last follow-up (range 8-25 months), 5 patients were alive, and 8 were lost to follow-up. Conclusion We present here a pediatric case of SOS due to stem cell transplant treated successfully with TIPS with a review of the literature. A timely, individualized application of TIPS can be effective in treating children with medication-refractory SOS.
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Affiliation(s)
- Lana Ramic
- From the Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada
| | - Matthew Speckert
- From the Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada
- Division of Hematology/Oncology, Children’s Hospital of Eastern Ontario, Ottawa, Ontario, Canada
| | - Raveena Ramphal
- Division of Hematology/Oncology, Children’s Hospital of Eastern Ontario, Ottawa, Ontario, Canada
| | - Simon C. Ling
- Division of Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Michael Temple
- Division of Diagnostic Imaging, St. Jude Children’s Research Hospital, TN
| | - Mohit Kehar
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Eastern Ontario, Ottawa, Ontario, Canada
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Hountondji L, Pureur D, Chabannes M, Felix S, Thévenot T. Transjugular Intrahepatic Portosystemic Shunt is effective to treat portal hypertension due to sinusoidal obstructive syndrome. Clin Res Hepatol Gastroenterol 2023; 47:102201. [PMID: 37643693 DOI: 10.1016/j.clinre.2023.102201] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/02/2023] [Revised: 08/24/2023] [Accepted: 08/25/2023] [Indexed: 08/31/2023]
Abstract
Transjugular Intrahepatic Portosystemic Shunt (TIPS) is an established procedure for the complications of portal hypertension, such as variceal bleeding, refractory ascites and hepatic hydrothorax. We report an original case of a renal transplant patient successfully treated with TIPS for portal hypertension due to sinusoidal obstructive syndrome (SOS) induced by azathioprine (AZA). By reporting this case, we wish to draw the attention of healthcare professionals managing organ transplant patients, especially nephrologists, to the possible occurrence of liver toxicity due to AZA, and to emphasize the role of TIPS as an effective therapeutic option for portal hypertension-related complications.
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Affiliation(s)
- Lina Hountondji
- Service d'Hépatologie et de Soins Intensifs Digestifs, Centre Hospitalier Universitaire Jean Minjoz, 25030 Besançon cedex, France.
| | - Dimitri Pureur
- Service d'Hépatologie et de Soins Intensifs Digestifs, Centre Hospitalier Universitaire Jean Minjoz, 25030 Besançon cedex, France
| | - Melchior Chabannes
- Service de Néphrologie dialyse et transplantation, Centre Hospitalier Universitaire Jean Minjoz, 25000 Besançon, France
| | - Sophie Felix
- Service d'Anatomo-pathologie, Centre Hospitalier Universitaire Jean Minjoz, 25000 Besançon, France
| | - Thierry Thévenot
- Service d'Hépatologie et de Soins Intensifs Digestifs, Centre Hospitalier Universitaire Jean Minjoz, 25030 Besançon cedex, France
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Li S, Li Y, Zhou C, Li H, Chen C, Peng C, Wang T, Liu F, Xiao J, Shi L. Transjugular Intrahepatic Portosystemic Shunt Benefits for Hepatic Sinusoidal Obstruction Syndrome Associated with Consumption of Gynura Segetum: a Propensity Score-Matched Analysis. Cardiovasc Intervent Radiol 2023:10.1007/s00270-023-03451-9. [PMID: 37225971 DOI: 10.1007/s00270-023-03451-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2022] [Accepted: 04/22/2023] [Indexed: 05/26/2023]
Abstract
PURPOSE Pyrrolidine alkaloids-related hepatic sinusoidal obstruction syndrome (PA-HSOS) is associated with a high mortality rate without standardized therapy. The efficacy of transjugular intrahepatic portosystemic shunts (TIPS) remains controversial. The study aimed to explore the risk factors influencing the clinical response in patients with PA-HSOS related to Gynura segetum (GS) to assess the disease prognosis at an early stage and to evaluate the efficacy of TIPS in these patients. METHODS This study retrospectively enrolled patients diagnosed with PA-HSOS between January 2014 and June 2021 with a clear history of exposure to GS. Univariate and multivariate logistic regression analyses were used to evaluate the risk factors influencing the clinical response in patients with PA-HSOS. Propensity score matched (PSM) was performed to compensate for differences in baseline characteristics between patients with and without TIPS. The primary outcome was the clinical response defined as the disappearance of ascites with normal total bilirubin levels and/or a reduction of elevated transaminase levels < 50% within 2 weeks. RESULTS A total of 67 patients were identified in our cohort with a clinical response rate of 58.2%. Of these, thirteen patients were assigned to the TIPS group and 54 to the conservative treatment group. Logistic regression analysis revealed that TIPS treatment (P = 0.047), serum globulin levels (P = 0.043), and prothrombin time (P = 0.001) were independent factors influencing clinical response. After PSM, there was a higher long-term survival rate of patients (92.3% vs. 51.3%, P = 0.021) and a shorter hospital stay (P = 0.043), but a high trend in hospital costs (P = 0.070) in the TIPS group. The 6-month survival probability in patients undergoing TIPS therapy was more than ninefold higher than in patients without receiving that treatment [hazard ratio (95% CI) = 9.304 (4.250, 13.262), P < 0.05]. CONCLUSIONS TIPS therapy may be an effective treatment option for patients with GS-related PA-HSOS.
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Affiliation(s)
- Sai Li
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Yong Li
- Department of Gastroenterology, Xiangya Hospital, Central South University, Changsha, 410005, Hunan, China
| | - Chunhui Zhou
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Haiping Li
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Changyong Chen
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Changli Peng
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Tianming Wang
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Fei Liu
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Juxiong Xiao
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China
| | - Liangrong Shi
- Interventional Radiology Center, Department of Radiology, Xiangya Hospital Central South University, Changsha, 410005, Hunan, China.
- Research Center for Geriatric Disorder, Xiangya Hospital Central South, Changsha, 410005, Hunan, China.
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Luo X, Nicoară-Farcău O, Magaz M, Betancourt F, Soy G, Baiges A, Turon F, Hernández-Gea V, García-Pagán JC. Obstruction of the liver circulation. CARDIO-HEPATOLOGY 2023:65-92. [DOI: 10.1016/b978-0-12-817394-7.00004-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Yoon JH, Choi CW, Won JH. Hepatic sinusoidal obstruction syndrome/veno-occlusive disease after hematopoietic cell transplantation: historical and current considerations in Korea. Korean J Intern Med 2021; 36:1261-1280. [PMID: 34555279 PMCID: PMC8588980 DOI: 10.3904/kjim.2021.082] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/10/2021] [Accepted: 04/28/2021] [Indexed: 12/14/2022] Open
Abstract
Hepatic sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a rare but severe complication of hematopoietic cell transplantation (HCT) showing high mortality. Multiple risk factors for SOS/VOD were identified, but it is often confused with other hepatic complications due to nonspecific clinical features. Therefore, diagnostic and severity criteria have been revised several times. The European Society of Blood and Marrow Transplantation suggested a new guideline that excludes the standard duration of development within 21 days, emphasizes late-onset SOS/VOD, and suggests the importance of Doppler ultrasonography. The severity criteria were further subdivided for guidance to begin active treatment using defibrotide which was approved in Korea since 2016. In a phase 3 trial, defibrotide had superior 100-day survival, compared to best available treatments (38.2% vs. 25.0%). Although several studies of SOS/VOD in Korean patients have been performed after the implementation of HCT, most involved small number of pediatric patients. Recently, the Korean Society of Blood and Marrow Transplantation investigated the incidence of SOS/VOD in the Korean population, and several influential studies of adult patients were published. Here, we summarize recent issues regarding the mechanism, diagnosis, severity criteria, prevention, and treatments of SOS/VOD in Korean patients, as well as recent analyses of nationwide incidence.
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Affiliation(s)
- Jae-Ho Yoon
- Department of Hematology, Catholic Hematology Hospital and Leukemia Research Institute, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul,
Korea
| | - Chul Won Choi
- Division of Hematology and Oncology, Department of Internal Medicine, Korea University Guro Hospital, Seoul,
Korea
| | - Jong-Ho Won
- Division of Hematology and Medical Oncology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul,
Korea
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10
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Deng L, Yin X, Zhao Y, Yang J, Yang H, Xu C, Li K. Outcomes of combination treatment with MARS and TIPS for hepatic veno-occlusive disease: a report of 12 cases. J Int Med Res 2021; 48:300060520980877. [PMID: 33372815 PMCID: PMC7783893 DOI: 10.1177/0300060520980877] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023] Open
Abstract
BACKGROUND In patients with acute liver injury caused by hepatic veno-occlusive disease (HVOD), molecular adsorbent recirculation system (MARS) may be used to improve liver function in conjunction with transjugular intrahepatic portosystemic shunt (TIPS) to reduce portal hypertension. METHODS Twelve patients were admitted to our hospital following treatment for HVOD for 10 to 21 days at other hospitals. All patients were treated with a combination of MARS and TIPS, and they were evaluated clinically including liver function tests. RESULTS After the initial treatment with MARS, liver function improved significantly in all patients. TIPS placement decreased the hepatic venous pressure gradient (HVPG) to 10.17 ± 2.26 mmHg from a pre-TIPS HVPG of 23.58 ± 9.43 mmHg. The outcomes of combination treatment with MARS and TIPS in 12 patients with HVOD were as follows: 1) improvement of various clinical and biological parameters leading to full recovery in 1 year in 6 patients; 2) full recovery following liver transplantation for acute liver failure in three patients; and 3) three patients died due to hepatic failure after TIPS placement. CONCLUSION The combination of MARS and TIPS creation is promising as a potential treatment for acute HVOD, and it showed an improvement in overall survival.
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Affiliation(s)
- Li Deng
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
| | - Xiuli Yin
- Department of Gastroenterology, Rongjun Hospital of Shandong
Province, Jinan, China
| | - Yingying Zhao
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
| | - Jing Yang
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
| | - Hongli Yang
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
| | - Changqing Xu
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
| | - Kun Li
- Department of Gastroenterology, Shandong Qianfoshan Hospital,
The First Affiliated Hospital of Shandong First Medical University, Jinan,
China
- Kun Li, Department of Gastroenterology,
Shandong Qianfoshan Hospital, The First Affiliated Hospital of Shandong First
Medical University, 16766 Jingshi Road, Jinan, Shandong Province 250014, China.
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11
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Magaz M, García-Pagán JC. Risk factors of poor prognosis in patients with pyrrolidine alkaloids induced hepatic sinusoidal obstruction syndrome after transjugular intrahepatic portosystemic shunt : Transjugular intrahepatic portosystemic shunt in patients with sinusoidal obstruction syndrome, etiology matters. Hepatol Int 2021; 15:568-570. [PMID: 33733320 DOI: 10.1007/s12072-021-10166-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Accepted: 02/22/2021] [Indexed: 11/28/2022]
Affiliation(s)
- Marta Magaz
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital ClínicInstitut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Villarroel 170, 08036, Barcelona, Spain.,CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas Y Digestivas), Barcelona, Spain.,Health Care Provider of the European Reference Network On Rare Liver Disorders (ERN-Liver), Paris, France
| | - Juan Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital ClínicInstitut de Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Villarroel 170, 08036, Barcelona, Spain. .,CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas Y Digestivas), Barcelona, Spain. .,Health Care Provider of the European Reference Network On Rare Liver Disorders (ERN-Liver), Paris, France.
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12
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Xiao J, Tu J, Zhang H, Zhang F, Zhang W, Xu H, Yin Q, Yang J, Han H, Wang Y, Zhang B, Peng C, Zou X, Zhang M, Zhuge Y. Risk factors of poor prognosis in patients with pyrrolidine alkaloid-induced hepatic sinusoidal obstruction syndrome after transjugular intrahepatic portosystemic shunt. Hepatol Int 2021; 15:720-729. [PMID: 33507485 DOI: 10.1007/s12072-020-10126-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2020] [Accepted: 12/19/2020] [Indexed: 11/09/2022]
Abstract
AIMS Transjugular intrahepatic portosystemic shunt (TIPS) is an effective method in treating patients with severe hepatic sinusoidal obstruction syndrome induced by pyrrolidine alkaloids (PA-HSOS). However, some patients still have poor postoperative prognosis. So, we aim to evaluate the predictors associated with poor outcomes in PA-HSOS patients receiving TIPS. METHODS Patients who were diagnosed as PA-HSOS and received TIPS in our hospital between January 2013 and April 2019 were reviewed retrospectively. Baseline information and clinical data were collected. The hazard ratios (HRs) of factors associated with poor prognosis were analyzed by Cox proportional hazard analysis. The Kaplan-Meier method was used to analyze and compare the cumulative incidence of the poor results and survival rate of patients. RESULTS During a median of 19.25-month follow-up, death occurred in 17 patients. We found that prothrombin time at baseline with an adjusted HR 1.110 (95% confidence interval 1.014-1.216, p = 0.024) and serum total bilirubin of 9 mg/dl 5 days after TIPS with an adjusted HR 1.114 (95% confidence interval 1.042-1.190, p = 0.001) were independent risk factors for death. The 1-year and 5-year survival rate were 86.2% and 82.1%, respectively. The 1-year survival rate in patients with prothrombin time > 17.85 s at baseline and serum total bilirubin > 9 mg/dl at 5 days after TIPS was significantly lower than that of patients below the corresponding threshold, respectively. CONCLUSIONS Prolonged prothrombin time at baseline and increased serum total bilirubin levels 5 days after TIPS are independent risk factors for predicting death after TIPS treatment in PA-HSOS patients.
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Affiliation(s)
- Jiangqiang Xiao
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Jingjing Tu
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Han Zhang
- Department of Gastroenterology, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, Nanjing, 210008, Jiangsu, China
| | - Feng Zhang
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Wei Zhang
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Hui Xu
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Qin Yin
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Jian Yang
- Department of Ultrasound, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China
| | - Hao Han
- Department of Ultrasound, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China
| | - Yi Wang
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Bin Zhang
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Chunyan Peng
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Xiaoping Zou
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Ming Zhang
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China
| | - Yuzheng Zhuge
- Department of Gastroenterology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321#, Zhongshan Road, Nanjing, 210008, Jiangsu, China.
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Zhang L, Li Q, Makamure J, Zhao D, Liu Z, Zheng C, Liang B. Transjugular intrahepatic portosystemic shunt for hepatic sinusoidal obstruction syndrome associated with consumption of Gynura segetum. BMC Gastroenterol 2021; 21:26. [PMID: 33423668 PMCID: PMC7798314 DOI: 10.1186/s12876-021-01599-7] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2020] [Accepted: 01/01/2021] [Indexed: 12/03/2022] Open
Abstract
Background To evaluate the efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) on hepatic sinusoidal obstruction syndrome (HSOS) associated with consumption of Gynura segetum (GS). Methods We retrospectively reviewed 9 consecutive patients with GS-related HSOS who were refractory to supportive treatment and underwent TIPS at our institution between January 2014 and September 2019. The patients were evaluated for safety and efficacy, including TIPS complications and changes in portosystemic pressure gradient (PPG), ascites, total bilirubin, liver size and portal vein diameter. Results TIPS procedures were performed successfully in the 9 patients, and no technically-related complications due to the TIPS procedure were recorded. The PPG was improved by TIPS in all patients (mean PPG before TIPS, 30.4 ± 5.2 vs. 13.0 ± 4.1 mm Hg post-TIPS, P = 0.008). One patient who was lost to follow-up, whereas the remaining 8 patients survived with a median follow-up period of 12 months (range 5–39 months). Although the total bilirubin was significantly increased 5–7 days after TIPS compared with that before the procedure (3.57 ± 1.58 vs. 4.82 ± 2.06 mg/dl, P = 0.017), it returned to baseline levels at 1-month follow-up (3.53 ± 2.72 vs. 4.82 ± 2.06 mg/dl, P = 0.401). The patients experienced complete resolution or noticeable reduction of ascites (P < 0.001), significant reduction of liver size (16.7 ± 2.2 vs. 13.7 ± 1.7 cm, P = 0.018), and significant enlargement of the portal trunk (10.7 ± 2.5 vs. 13.4 ± 2.4 mm, P = 0.017) after TIPS compared to the pre-TIPS state. Conclusion TIPS may offer a potentially useful treatment for the GS-related HSOS.
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Affiliation(s)
- Lijie Zhang
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Qing Li
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Joyman Makamure
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Dan Zhao
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Ziyi Liu
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Chuansheng Zheng
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China
| | - Bin Liang
- Department of Radiology, Hubei Key Laboratory of Molecular Imaging, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Road, Wuhan, 430022, China.
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De La Pinta Alonso C. Radiation-induced liver disease in the era of SBRT: a review. Expert Rev Gastroenterol Hepatol 2020; 14:1195-1201. [PMID: 32886888 DOI: 10.1080/17474124.2020.1814744] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2020] [Accepted: 08/21/2020] [Indexed: 10/23/2022]
Abstract
INTRODUCTION Stereotactic Body Radiotherapy (SBRT) in liver tumors allows ablative radiation doses in tumors preserving the liver tissue. However, liver is a parallel organ allowing high doses in a small region to preserve its function. If this is not possible, radio-induced liver toxicity is produced. Radio-induced liver toxicity or radio-induced liver disease (RILD) is the most serious toxicity in liver radiotherapy. AREAS COVERED In this review, we analyzed published literature on PubMed and MEDLINE. We included papers in English language with information about RILD characteristics, diagnostic, risk factors, pathophysiology, and treatment. All citations were evaluated for relevant content and validation. EXPERT OPINION The study of RILD is fundamental before the implementation of liver SBRT. Radio-induced liver toxicity is a complication that can be fatal for patients. This is a diagnosis of exclusion and it is essential that experts in the treatment of hepatic SBRT know about it and anticipate its development. The study and development of molecular or imaging biomarkers could be key in their diagnosis and prevention.
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Gómez-Centurión I, Bailén R, Oarbeascoa G, Muñoz C, Luque AÁ, Boyra ME, Calleja E, Rincón D, Dorado N, Barzallo P, Anguita J, Díez-Martín JL, Kwon M. Transjugular Intrahepatic Portosystemic Shunt for Very Severe Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome (VOD/SOS) after Unmanipulated Haploidentical Hematopoietic Stem Cell Transplantation with Post-transplantation Cyclophosphamide. Biol Blood Marrow Transplant 2020; 26:2089-2097. [PMID: 32791193 DOI: 10.1016/j.bbmt.2020.08.006] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2020] [Revised: 07/13/2020] [Accepted: 08/03/2020] [Indexed: 12/12/2022]
Abstract
Hepatic veno-occlusive disease or sinusoidal obstruction syndrome (VOD/SOS) is a threatening complication after both autologous and allogeneic hematopoietic stem cell transplantation (HSCT), with high mortality rates despite early medical treatment, including the use of defibrotide (DF). We retrospectively analyzed 185 unmanipulated haploidentical (haplo-) HSCT with post-transplantation cyclophosphamide as graft-versus-host disease prophylaxis performed consecutively between 2011 and June 2019 in a single center. Seventeen patients (9.2%) were diagnosed with VOD/SOS. Based on revised European Society for Blood and Marrow Transplantation severity criteria, the VOD/SOS cases were classified as mild in 2 patients (11.7%), moderate in 2 (11.7%), severe in 2 (11.7%), and very severe in 11 (64.9%). Thirteen patients (76%) were treated with DF, including all patients with severe or very severe VOD/SOS, except 1 patient with CNS hemorrhage. Sixteen patients (94%) were alive at day +100 after HSCT. Seven patients (41%) with very severe VOD/SOS were treated with transjugular intrahepatic portosystemic shunt (TIPS) owing to rapid clinical or analytical deterioration or refractory hepatorenal syndrome despite medical treatment, including DF. TIPS insertion was performed at a median time since VOD/SOS diagnosis of 4 days (range, 1 to 28 days) without technical complications in any case. The median hepatic venous pressure gradient before and after TIPS treatment was 24 mmHg (range, 14 to 29 mmHg) and 7 mmHg (range, 2 to 11 mmHg), respectively, with a median drop of 16 mmHg (range, 9 to 19 mmHg). Following TIPS insertion, all patients showed clinical improvement with hepatomegaly, ascites, and renal failure resolution, and all showed analytical improvement with reduced alanine aminotransferase (ALT), creatinine, and international normalized ratio values, except for patient 2, whose indication for TIPS was refractory hepatorenal syndrome with a normal ALT level. The 6 patients who had initiated DF before TIPS insertion completed 21 days of treatment. All patients met the criteria for complete remission (CR) at a median of 8 days after TIPS insertion (range, 2 to 82 days). The 100-day overall survival was 100%. For patients with rapid progressive VOD/SOS, early TIPS insertion allowed completion of DF therapy. The use of TIPS together with DF resulted in CR and no associated complications with no VOD/SOS-associated mortality despite high severity. In our experience, timely and individualized use of TIPS significantly improves outcomes of very severe VOD/SOS after haplo-HSCT. Therefore, TIPS should be promptly considered in rapidly progressive cases.
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Affiliation(s)
| | - Rebeca Bailén
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Gillen Oarbeascoa
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Cristina Muñoz
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Arturo Álvarez Luque
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Miguel Echenagusia Boyra
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Interventional Radiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Enrique Calleja
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Interventional Radiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Diego Rincón
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Department of Hepatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Nieves Dorado
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Paola Barzallo
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Javier Anguita
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - José Luis Díez-Martín
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain; Department of Hematology, Universidad Complutense de Madrid, Madrid, Spain
| | - Mi Kwon
- Department of Hematology, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
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Zhou CZ, Wang RF, Lv WF, Fu YQ, Cheng DL, Zhu YJ, Hou CL, Ye XJ. Transjugular intrahepatic portosystemic shunt for pyrrolizidine alkaloid-related hepatic sinusoidal obstruction syndrome. World J Gastroenterol 2020; 26:3472-3483. [PMID: 32655270 PMCID: PMC7327784 DOI: 10.3748/wjg.v26.i24.3472] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2020] [Revised: 04/30/2020] [Accepted: 05/15/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Treatments for hepatic sinusoidal obstruction syndrome (HSOS) are limited.
AIM To evaluate transjugular intrahepatic portosystemic shunting (TIPS) as a treatment for pyrrolidine alkaloid-related HSOS (PA-HSOS).
METHODS This retrospective analysis included patients with PA-HSOS admitted to the First Affiliated Hospital of the University of Science and Technology of China (June 2015 to January 2019). Baseline clinical characteristics and follow-up data were extracted from the medical records. All patients included in this study experienced failure of initial therapy. Patients were divided into the TIPS and conservative treatment groups according to the therapy they received. Liver function, maximal ascites depth, imaging characteristics, pathology findings, and survival were compared between groups.
RESULTS The TIPS group included 37 patients (28 males), and the conservative treatment group included 17 patients (11 males). Baseline characteristics were similar between groups. There were two deaths in the TIPS group and seven deaths in the conservative treatment group during follow-up (3-48 mo). The 3-, 6-, 12- and 24-mo survival rates were 94.6%, 94.6%, 94.6% and 94.6%, respectively, in the TIPS group and 70.6%, 57.8%, 57.8% and 57.8%, respectively, in the conservative treatment group. Kaplan-Meier analysis revealed significantly longer survival for the TIPS group than for the conservative treatment group (P = 0.001). Compared with the pre-treatment value, maximal ascites depth was significantly lower at 1 wk, 2 wk, 1 mo, and 3 mo for the TIPS group (all P < 0.05) but not in the conservative treatment group. Contrast-enhanced computed tomography demonstrated the disappearance of patchy liver enhancement after TIPS. Pathology showed that liver congestion and hepatocyte swelling improved with time after TIPS placement.
CONCLUSION TIPS may achieve better outcomes than conventional symptomatic treatment in patients with PA-HSOS.
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Affiliation(s)
- Chun-Ze Zhou
- Interventional Radiology, Medical College of Shandong University, Jinan 250021, Shandong Province, China
- Interventional Radiology Department, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Rui-Feng Wang
- Department of Nephrology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250033, Shandong Province, China
| | - Wei-Fu Lv
- Interventional Radiology, Medical College of Shandong University, Jinan 250021, Shandong Province, China
- Interventional Radiology Department, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Yu-Qin Fu
- Department of Nephrology, The Second Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250033, Shandong Province, China
| | - De-Lei Cheng
- Interventional Radiology Department, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Yi-Jiang Zhu
- Interventional Radiology Department, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Chang-Long Hou
- Interventional Radiology Department, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
| | - Xian-Jun Ye
- Department of Ultrasound, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, Anhui Province, China
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Prophylactic, preemptive, and curative treatment for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a position statement from an international expert group. Bone Marrow Transplant 2019; 55:485-495. [PMID: 31576023 PMCID: PMC7051913 DOI: 10.1038/s41409-019-0705-z] [Citation(s) in RCA: 65] [Impact Index Per Article: 10.8] [Reference Citation Analysis] [Abstract] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2019] [Accepted: 06/06/2019] [Indexed: 02/06/2023]
Abstract
Sinusoidal obstruction syndrome, also known as veno-occlusive disease (SOS/VOD), is a potentially life-threatening complication that can develop after hematopoietic cell transplantation (HCT). While SOS/VOD may resolve within a few weeks in the majority of patients with mild-to-moderate disease, the most severe forms result in multiorgan dysfunction and are associated with a high mortality rate (>80%). Therefore, careful surveillance may allow early detection of SOS/VOD, particularly as the licensed available drug is proven to be effective and reduce mortality. The aim of this work is to propose an international consensus guideline for the treatment and prevention of SOS/VOD in adult patients, on behalf of an international expert group.
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Ravaioli F, Colecchia A, Alemanni LV, Vestito A, Dajti E, Marasco G, Sessa M, Pession A, Bonifazi F, Festi D. Role of imaging techniques in liver veno-occlusive disease diagnosis: recent advances and literature review. Expert Rev Gastroenterol Hepatol 2019; 13:463-484. [PMID: 30895833 DOI: 10.1080/17474124.2019.1588111] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Veno-occlusive-disease (VOD), known also as sinusoidal-obstruction-syndrome (SOS), is one of the main complications of haematopoietic stem cell transplantation and is related to the treatment with pyrrolizidine alkaloids or other toxic agents (chemotherapy for liver-metastasis). Clinical diagnosis using the recent criteria from the European Society for Blood and Marrow Transplantation, is the reference for VOD/SOS diagnosis. However, increasing evidence suggests the emerging role of several imaging methods that could help the clinician in VOD/SOS assessment. Areas covered: This review evaluates the current literature on the various imaging techniques used in VOD/SOS diagnosis in several clinical scenarios. Literature searches were performed using several keywords on MEDLINE/Ovid/In-Process/Cochrane Library/EMBASE and PubMed up to July 2018. Expert commentary: Hepatic-gradient-measurement (HVPG) and contextual transjugular-liver-biopsy are invasive and should always be considered in unclear cases. The main studies revolve around ultrasound with Doppler evaluation, identifying numerous findings suggestive of VOD/SOS. However, their accuracy and validation are still suboptimal and controversial. CT-Scan and MRI have shown encouraging data in other contexts in which VOD/SOS can develop, but studies on the post-HSCT patient are lacking. Elastography techniques measuring liver stiffness (LSM) represent the most recent and promising approach for an accurate and early diagnosis of VOD/SOS. In our view, a multidisciplinary approach to the VOD/SOS diagnosis should be highly encouraged.
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Affiliation(s)
- Federico Ravaioli
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
| | - Antonio Colecchia
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy.,b Gastroenterology Unit , Borgo Trento University Hospital , Verona , Italy
| | | | - Amanda Vestito
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
| | - Elton Dajti
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
| | - Giovanni Marasco
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
| | - Mariarosaria Sessa
- c Stem Cell Transplant Program , Institute of Haematology "Seragnoli", University Hospital Sant'Orsola Malpighi , Bologna , Italy
| | - Andrea Pession
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
| | - Francesca Bonifazi
- c Stem Cell Transplant Program , Institute of Haematology "Seragnoli", University Hospital Sant'Orsola Malpighi , Bologna , Italy
| | - Davide Festi
- a Department of Medical and Surgical Sciences (DIMEC) , University of Bologna , Bologna , Italy
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Zhuge Y, Liu Y, Xie W, Zou X, Xu J, Wang J. Expert consensus on the clinical management of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome. J Gastroenterol Hepatol 2019; 34:634-642. [PMID: 30669184 DOI: 10.1111/jgh.14612] [Citation(s) in RCA: 65] [Impact Index Per Article: 10.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2018] [Revised: 12/28/2018] [Accepted: 01/17/2019] [Indexed: 02/06/2023]
Abstract
Hepatic sinusoidal obstruction syndrome (HSOS) is a hepatic vascular disease presenting with abdominal distension, pain in the hepatic region, ascites, jaundice, and hepatomegaly. In China, this disease is often associated with the oral intake of plants that contain pyrrolidine alkaloids. The existing guidelines are limited to HSOS associated with hematopoietic stem cell transplantation in Western countries. The Hepatobiliary Diseases Committee of the Chinese Society of Gastroenterology convened an expert consensus conference on the diagnosis and treatment of PA-HSOS to evaluate current research in China and abroad. The "Nanjing criteria" developed by the committee to diagnose PA-HSOS include a confirmed history of PA-containing plant use and (i) abdominal distention and/or pain in the hepatic region, hepatomegaly, and ascites; (ii) elevation of serum total bilirubin or abnormal laboratory liver tests; (iii) evidence on enhanced computed tomography or magnetic resonance imaging; or (iv) pathological evidence that rules out other known causes of liver injury. Supportive symptomatic treatment, anticoagulant therapy, and placement of a transjugular intrahepatic portosystemic shunt for patients who do not respond to medical treatment are effective for the treatment of PA-HSOS. The benefits of glucocorticoids and prostaglandin E1 in PA-HSOS are not clear.
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Affiliation(s)
- Yuzheng Zhuge
- Department of Gastroenterology, Nanjing Drum Tower Hospital, Affiliated to Nanjing University Medical School, Nanjing, China
| | - Yulan Liu
- Department of Gastroenterology, Peking University People's Hospital, Beijing, China
| | - Weifen Xie
- Department of Gastroenterology, Changzheng Hospital of the Second Military Medical University, Shanghai, China
| | - Xiaoping Zou
- Department of Gastroenterology, Nanjing Drum Tower Hospital, Affiliated to Nanjing University Medical School, Nanjing, China
| | - Jianming Xu
- Department of Gastroenterology, The First Affiliated Hospital of Anhui University, Anhui, China
| | - Jiyao Wang
- Department of Gastroenterology, Zhongshan Hospital of Fudan University, Shanghai, China
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Nathan S, Ustun C. Complications of Stem Cell Transplantation that Affect Infections in Stem Cell Transplant Recipients, with Analogies to Patients with Hematologic Malignancies. Infect Dis Clin North Am 2019; 33:331-359. [PMID: 30940464 DOI: 10.1016/j.idc.2019.01.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
This article discusses the complications of hematopoietic stem cell transplantion (HSCT) that affect infections in HSCT recipients, with analogies to patients with hematologic malignancies. Mucositis, with mucosal barrier disruption, is common and increases the risk of gram-positive and anaerobic bacterial, and fungal infections, and can evolve to typhlitis. Engraftment syndrome; graft-versus-host disease, hepatic sinusoidal obstruction syndrome; and posterior reversible encephalopathy syndrome can affect the infectious potential either directly from organ dysfunction or indirectly from specific treatment. Pulmonary infections can predispose to life threatening complications including diffuse alveolar hemorrhage, idiopathic pulmonary syndrome, bronchiolitis obliterans syndrome, and bronchiolitis obliterans with organizing pneumonia.
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Affiliation(s)
- Sunita Nathan
- Section of Bone Marrow Transplant and Cellular Therapy, Division of Hematology, Oncology and Cell Therapy, Rush University Medical Center, 1725 West Harrison Street, Suite 809, Chicago, IL 60612, USA
| | - Celalettin Ustun
- Section of Bone Marrow Transplant and Cellular Therapy, Division of Hematology, Oncology and Cell Therapy, Rush University Medical Center, 1725 West Harrison Street, Suite 809, Chicago, IL 60612, USA.
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Ferrusquía-Acosta J, Hernández-Gea V. TIPS Indications and Contraindications—Pushing the Limits: Is Earlier Better? CURRENT HEPATOLOGY REPORTS 2019; 18:87-95. [DOI: 10.1007/s11901-019-00453-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Wang Y, Qiao D, Li Y, Xu F. Risk factors for hepatic veno-occlusive disease caused by Gynura segetum: a retrospective study. BMC Gastroenterol 2018; 18:156. [PMID: 30367628 PMCID: PMC6204041 DOI: 10.1186/s12876-018-0879-7] [Citation(s) in RCA: 33] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2018] [Accepted: 10/02/2018] [Indexed: 12/11/2022] Open
Abstract
Background Hepatic veno-occlusive disease (HVOD) caused by Gynura segetum has been increasingly reported in China in recent years. The aim of this retrospective study was to identify independent prognostic markers for survival in patients with Gynura segetum-induced HVOD and to evaluate the effect of anticoagulants and transjugular intrahepatic portosystemic shunt (TIPS) on survival rate. Methods Clinical data including symptoms, signs, imaging characteristics, laboratory test results, results of liver tissue biopsies, type of treatment during follow-up and clinical outcomes were collected. Univariate, multivariate and time-dependent Cox regression analyses were performed. Results Survival rates were 91% (95% confidence interval [CI], 82–95%), 64% (95% CI, 53–69%) and 57% (95% CI, 51–65%) at 1, 3 and 60 months, respectively. Total bilirubin, albumin and hepatic encephalopathy were independent prognostic markers of survival. Anticoagulants were administered to 76% of the patients. Among 75 patients treated with anticoagulants, 49 patients (65.3%) were cured, whereas 26 patients (34.7%) died; the cure rate in anticoagulant-treated patients was higher than that of those not treated with anticoagulants (χ2 = 9.129, P = 0.004). Cure rate of the anticoagulation + TIPS treatment group was 64.3%, which was also higher than that of the non-anticoagulation group; however, this was not significantly different (χ2 = 3.938, P = 0.096). Conclusions The presence of hepatic encephalopathy, serum bilirubin and albumin levels were major prognostic factors for Gynura segetum-induced HVOD. Anticoagulation therapy significantly increased the cure rate; however, TIPS treatment did not have a beneficial effect on the cure rate.
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Affiliation(s)
- Yan Wang
- Department of Gastroenterology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Donglu, Zhengzhou, 450052, Henan, China
| | - Dan Qiao
- Department of Zhengzhou Center for Disease Control and Prevention, Zhengzhou, China
| | - Ya Li
- Department of Gastroenterology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Donglu, Zhengzhou, 450052, Henan, China
| | - Feng Xu
- Department of Gastroenterology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Donglu, Zhengzhou, 450052, Henan, China.
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Zhuge YZ, Wang Y, Zhang F, Zhu CK, Zhang W, Zhang M, He Q, Yang J, He J, Chen J, Zou XP. Clinical characteristics and treatment of pyrrolizidine alkaloid-related hepatic vein occlusive disease. Liver Int 2018; 38:1867-1874. [PMID: 29297975 DOI: 10.1111/liv.13684] [Citation(s) in RCA: 53] [Impact Index Per Article: 7.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/07/2017] [Accepted: 12/11/2017] [Indexed: 12/21/2022]
Abstract
BACKGROUND AND AIMS Hematopoietic stem cell transplantation related hepatic vein occlusive disease (HSCT-HVOD) has been researched extensively; however, little is known about the clinical features and treatment of pyrrolizidine alkaloid-induced HVOD (PA-HVOD). This retrospective single centre study examined the clinical and laboratory characteristics of 108 patients with acute PA-HVOD and explored the efficacy of anticoagulation and TIPS therapy. METHODS The study included 108 consecutive patients with PA-HVOD between July 2008 and June 2016. The clinical manifestations and the results of laboratory and imaging tests were evaluated. The survival rates of patients treated with different approaches were recorded. RESULTS Serum total bilirubin was <34.2 μmol/L (2 mg/dL) in approximately 40% of patients. More than 90% of patients were presented with hepatomegaly, uneven liver perfusion in the balance phase, compressive stenosis of the hepatic segmental inferior vena cava and decreased peak velocity of portal vein blood flow. Severe portal hypertension was observed in all patients undergoing HVPG examination or TIPS operation. Anticoagulation therapy with low molecular weight heparin combined with warfarin was significantly more effective than liver protection and supportive therapy, and TIPS further improved the prognosis of patients who did not respond to anticoagulation therapy. The total effective rate of the anticoagulation-TIPS ladder therapeutic strategy was 91%. CONCLUSIONS Patients with PA-HVOD had different characteristics than those with HSCT-HVOD. Anticoagulation and TIPS treatment may be effective for patients with PA-HVOD.
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Affiliation(s)
- Yu Z Zhuge
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Yi Wang
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Feng Zhang
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Cheng K Zhu
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Wei Zhang
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Ming Zhang
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Qibin He
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Jian Yang
- Department of Ultrasound Diagnosis, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Jian He
- Department of Radiology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Jun Chen
- Department of Pathology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
| | - Xiao P Zou
- Department of Gastroenterology, Drum Tower Hospital, Nanjing University School of Medicine, Nanjing, China
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Direct Intrahepatic Portocaval Shunt for Sinusoidal Obstruction Syndrome Associated with Hepatotoxicity of Pyrrolizidine Alkaloids. BIOMED RESEARCH INTERNATIONAL 2018; 2018:9804582. [PMID: 30009181 PMCID: PMC6020549 DOI: 10.1155/2018/9804582] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/10/2017] [Revised: 03/20/2018] [Accepted: 04/18/2018] [Indexed: 02/07/2023]
Abstract
We retrospectively identified 89 consecutive patients from January 2004 to January 2012 to investigate efficacy of direct intrahepatic portocaval shunt (DIPS) combined with inferior vena cava (IVC) stenting for sinusoidal obstruction syndrome (SOS) associated with hepatotoxicity of pyrrolizidine alkaloids. Indications for treatment were variceal hemorrhage and/or refractory ascites. Patients were treated with DIPS plus IVC stenting (group A, n=68) or DIPS alone (group B, n=21). A technical success rate of 100% was obtained in all 89 patients, and there were no early procedure-related adverse events or 30-day mortality. Mean portosystemic gradient decreased in both groups. Changes in aspartate and alanine aminotransferases and total bilirubin did not differ between the groups. Ascites disappeared in group A but was not obvious in group B until IVC stenting. During follow-up, recurrent bleeding and ascites and incidence of hepatic encephalopathy did not differ between the groups. The 1-, 3-, and 5-year survival rate was 98, 89.59, and 80%, respectively. Satisfactory clinical results were obtained for combined DIPS and IVC stenting for SOS associated with pyrrolizidine-alkaloid-related decompensated cirrhosis.
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25
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Martín-Llahí M, Albillos A, Bañares R, Berzigotti A, García-Criado MÁ, Genescà J, Hernández-Gea V, Llop-Herrera E, Masnou-Ridaura H, Mateo J, Navascués CA, Puente Á, Romero-Gutiérrez M, Simón-Talero M, Téllez L, Turon F, Villanueva C, Zarrabeitia R, García-Pagán JC. Enfermedades vasculares del hígado. Guías Clínicas de la Sociedad Catalana de Digestología y de la Asociación Española para el Estudio del Hígado. GASTROENTEROLOGIA Y HEPATOLOGIA 2017; 40:538-580. [PMID: 28610817 DOI: 10.1016/j.gastrohep.2017.03.011] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/13/2017] [Accepted: 03/29/2017] [Indexed: 12/11/2022]
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Abstract
Radioembolization (RE) is a relatively novel treatment modality for primary and secondary hepatic malignancies. Microspheres embedded with a β-emitting radioisotope are injected into the hepatic artery, resulting in microsphere deposition in the tumor arterioles and normal portal triads. Microsphere deposition in nontumorous parenchyma can result in radiation-induced liver injury, with lethal RE-induced liver disease (REILD) at the outer end of the spectrum. The primary aim of this study was to evaluate RE-related hepatotoxicity and present an overview of the currently applied definitions and clinically relevant characteristics of REILD. A systematic literature search on REILD was performed. Studies after the introduction of the term REILD (2008) were screened for definitions of REILD. Hepatotoxicity and applied definitions of REILD were compared. Liver biochemistry test abnormalities occur in up to 100% of patients after RE, mostly self-limiting. The incidence of symptomatic REILD varied between 0 and 31%, although in most reports, the incidence was 0-8%, with a lethal outcome in 0-5%. With the exception of bilirubin, the presentation of hepatotoxicity and REILD was similar for cirrhotic and noncirrhotic patients. No uniform definition of REILD was established in the current literature. Here, we propose a unifying definition and grading system for REILD. RE-related hepatotoxicity is a common phenomenon; symptomatic REILD, however, is rare. Currently, reporting of REILD is highly variable, precluding reliable comparison between studies, identification of risk factors, and treatment developments.
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27
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An overview of hematopoietic stem cell transplantation related thrombotic complications. Crit Rev Oncol Hematol 2016; 107:149-155. [DOI: 10.1016/j.critrevonc.2016.09.004] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2016] [Revised: 06/19/2016] [Accepted: 09/21/2016] [Indexed: 02/07/2023] Open
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Sinusoidal obstruction syndrome/veno-occlusive disease: current situation and perspectives-a position statement from the European Society for Blood and Marrow Transplantation (EBMT). Bone Marrow Transplant 2015; 50:781-9. [PMID: 25798682 PMCID: PMC4456788 DOI: 10.1038/bmt.2015.52] [Citation(s) in RCA: 273] [Impact Index Per Article: 27.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2014] [Accepted: 01/12/2015] [Indexed: 12/13/2022]
Abstract
Sinusoidal obstruction syndrome or veno-occlusive disease (SOS/VOD) is a potentially life-threatening complication of hematopoietic SCT (HSCT). This review aims to highlight, on behalf of the European Society for Blood and Marrow Transplantation, the current knowledge on SOS/VOD pathophysiology, risk factors, diagnosis and treatments. Our perspectives on SOS/VOD are (i) to accurately identify its risk factors; (ii) to define new criteria for its diagnosis; (iii) to search for SOS/VOD biomarkers and (iv) to propose prospective studies evaluating SOS/VOD prevention and treatment in adults and children.
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29
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Fan CQ, Crawford JM. Sinusoidal obstruction syndrome (hepatic veno-occlusive disease). J Clin Exp Hepatol 2014; 4:332-46. [PMID: 25755580 PMCID: PMC4298625 DOI: 10.1016/j.jceh.2014.10.002] [Citation(s) in RCA: 182] [Impact Index Per Article: 16.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2014] [Accepted: 10/20/2014] [Indexed: 12/12/2022] Open
Abstract
Hepatic sinusoidal obstruction syndrome (SOS) is an obliterative venulitis of the terminal hepatic venules, which in its more severe forms imparts a high risk of mortality. SOS, also known as veno-occlusive disease (VOD), occurs as a result of cytoreductive therapy prior to hematopoietic stem cell transplantation (HSCT), following oxaliplatin-containing adjuvant or neoadjuvant chemotherapy for colorectal carcinoma metastatic to the liver and treated by partial hepatectomy, in patients taking pyrrolizidine alkaloid-containing herbal remedies, and in other particular settings such as the autosomal recessive condition of veno-occlusive disease with immunodeficiency (VODI). A central pathogenic event is toxic destruction of hepatic sinusoidal endothelial cells (SEC), with sloughing and downstream occlusion of terminal hepatic venules. Contributing factors are SEC glutathione depletion, nitric oxide depletion, increased intrahepatic expression of matrix metalloproteinases and vascular endothelial growth factor (VEGF), and activation of clotting factors. The clinical presentation of SOS includes jaundice, development of right upper-quadrant pain and tender hepatomegaly, ascites, and unexplained weight gain. Owing to the potentially critical condition of these patients, transjugular biopsy may be the preferred route for liver biopsy to exclude other potential causes of liver dysfunction and to establish a diagnosis of SOS. Treatment includes rigorous fluid management so as to avoid excessive fluid overload while avoiding too rapid diuresis or pericentesis, potential use of pharmaceutics such as defibrotide, coagulolytic agents, or methylprednisolone, and liver transplantation. Proposed strategies for prevention and prophylaxis include reduced-intensity conditioning radiation for HSCT, treatment with ursodeoxycholic acid, and inclusion of bevacizumab with oxaliplatin-based chemotherapeutic regimes. While significant progress has been made in understanding the pathogenesis of SOS and in mitigating against its adverse outcomes, this condition remains a serious complication of a selective group of medical treatments.
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Key Words
- AML, acute myeloid leukemia
- APRI, aspartate aminotransferase to platelet ratio
- AST, aspartate aminotransferase
- Bmab, bevacizumab
- Colorectal cancer
- DF, defibrotide
- FOLFOX, chemotherapy regimen containing Folinic acid, 5-Fluorouracil, and Oxaliplatin
- GO, gemtuzumab ozogamicin
- GSTM1, glutathione S-transferase M1
- GVHD, graft-versus-host disease
- HSCT, hematopoietic stem cell transplantation
- Hematopoietic stem cell transplantation
- Herbal remedies
- Liver
- MOF, multi-organ failure
- Oxaliplatin
- PML, promyelocytic leukemia protein
- RIC-HSCT, reduced-intensity conditioning hematopoietic stem cell transplantation
- RILD, radiation-induced liver disease
- RT, radiation therapy
- SEC, sinusoidal endothelial cells
- SOS, sinusoidal obstruction syndrome
- TBI, total body irradiation
- TIPS, transjugular intrahepatic porto-systemic shunt
- UPLC-MS, ultra-performance liquid chromatography-mass spectrometry
- V-PYRRO/NO, O(2)-vinyl 1-(pyrrolidin-1-yl)diazen-1-ium-1,2-diolate
- VEGF, vascular endothelial growth factor
- VEGFR, vascular endothelial growth factor receptor
- VOD, veno-occlusive disease
- VODI, veno-occlusive disease with immunodeficiency
- l-NAME, N(G)-nitro-l-arginine methyl ester
- s-ICAM-1, soluble intercellular adhesion molecular-1
- t-PA, tissue plasminogen activator
- v-WF, von Willebrand factor
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Affiliation(s)
| | - James M. Crawford
- Address for correspondence: James M. Crawford, North Shore-LIJ Laboratories, 10 Nevada Drive, Lake Success, NY 11042-1114, USA. Tel.: +1 516 719 1060; fax: +1 516 719 1062.
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Carreras E. How I manage sinusoidal obstruction syndrome after haematopoietic cell transplantation. Br J Haematol 2014; 168:481-91. [PMID: 25401997 DOI: 10.1111/bjh.13215] [Citation(s) in RCA: 109] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2014] [Accepted: 10/15/2014] [Indexed: 12/24/2022]
Abstract
Sinusoidal obstruction syndrome (SOS), also called veno-occlusive disease of the liver, is one of the most relevant complications of endothelial origin that appears early after haematopoietic cell transplantation (HCT). Despite its relatively low incidence and the fact that most cases of SOS resolve spontaneously, the cases that evolve to multi-organ failure (MOF; severe SOS) have a mortality rate higher than 80% and represent one of the major clinical problems after HCT. For this reason, transplantation teams must have a pre-established policy regarding preventive measures in high-risk patients, strict daily control of weight and fluid balance during HCT, homogeneous diagnostic criteria, appropriate complementary studies for a correct differential diagnosis and measures to prevent and manage hepatorenal syndrome; in addition they must also be ready to start early treatment with defibrotide in patients with a possible severe SOS. Due to the lack of definitive evidence to enable the establishment of general recommendations in the management of SOS, this review analyses all of these aspects based on the author's personal experience.
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Affiliation(s)
- Enric Carreras
- Haematology Department, Hospital Clinic, Barcelona, Spain; Spanish Bone Marrow Donor Program, Josep Carreras Foundation, Barcelona, Spain; Josep Carreras Leukaemia Research Institute, Barcelona, Spain
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Abstract
Transjugular intrahepatic portosystemic shunt (TIPS) is an established procedure for the complications of portal hypertension. The largest body of evidence for its use has been supported for recurrent or refractory variceal bleeding and refractory ascites. Its use has also been advocated for acute variceal bleed, hepatic hydrothorax, and hepatorenal syndrome. With the replacement of bare metal stents with polytetrafluoroethylene-covered stents, shunt patency has improved dramatically, thus, improving outcomes. Therefore, reassessment of its utility, management of its complications, and understanding of various TIPS techniques is important.
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Affiliation(s)
- Kavish R Patidar
- Department of Internal Medicine, Virginia Commonwealth University Hospital, 1200 East Broad Street, MCV Box 980342, Richmond, VA 23298-0342, USA
| | - Malcolm Sydnor
- Radiology, Virginia Commonwealth University Hospital, 1200 East Broad Street, MCV Box 980615, Richmond, VA 23298-0615, USA; Surgery, Virginia Commonwealth University Hospital, 1200 East Broad Street, Richmond, VA 23298, USA; Vascular Interventional Radiology, Virginia Commonwealth University Hospital, 1200 East Broad Street, Richmond, VA 23298, USA
| | - Arun J Sanyal
- Division of Gastroenterology, Department of Internal Medicine, Virginia Commonwealth University School of Medicine, 1200 East Broad Street, MCV Box 980342, Richmond, VA 23298-0342, USA.
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Copelan A, Kapoor B, Sands M. Transjugular intrahepatic portosystemic shunt: indications, contraindications, and patient work-up. Semin Intervent Radiol 2014; 31:235-42. [PMID: 25177083 DOI: 10.1055/s-0034-1382790] [Citation(s) in RCA: 79] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
The transjugular intrahepatic portosystemic shunt (TIPS) procedure is effective in achieving portal decompression and in managing some of the major complications of portal hypertension. While many clinicians are familiar with the two most common indications for TIPS placement, secondary prophylaxis of esophageal variceal hemorrhage and treatment of refractory ascites, evidence for its usefulness is growing in other entities, where it has been less extensively studied but demonstrates promising results. Newer indications include early utilization in the treatment of esophageal variceal hemorrhage, Budd-Chiari syndrome, ectopic varices, and portal vein thrombosis. The referring clinician and interventionist must remain cognizant of the contraindications to the procedure to avoid complications and potential harm to the patient. This review is designed to provide an in-depth analysis of the most common as well as less typical indications for TIPS placement, and to discuss the contraindications and appropriate patient evaluation for this procedure.
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Affiliation(s)
- Alexander Copelan
- Diagnostic Radiology Department, William Beaumont Hospital, Royal Oak, Michigan
| | | | - Mark Sands
- Imaging Institute, Cleveland Clinic, Cleveland, Ohio
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Abstract
In the 25 years since the first TIPS intervention has been performed, technical standards, indications, and contraindications have been set up. The previous considerable problem of shunt failure by thrombosis or intimal proliferation in the stent or in the draining hepatic vein has been reduced considerably by the availability of polytetrafluoroethylene (PTFE)-covered stents resulting in reduced rebleeding and improved survival. Unfortunately, most clinical studies have been performed prior to the release of the covered stent and, therefore, do not represent the present state of the art. In spite of this, TIPS has gained increasing acceptance in the treatment of the various complications of portal hypertension and vascular diseases of the liver.
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Affiliation(s)
- Martin Rössle
- Praxiszentrum and University Hospital, Freiburg, Germany.
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Loffroy R, Estivalet L, Cherblanc V, Favelier S, Pottecher P, Hamza S, Minello A, Hillon P, Thouant P, Lefevre PH, Krausé D, Cercueil JP. Transjugular intrahepatic portosystemic shunt for the management of acute variceal hemorrhage. World J Gastroenterol 2013; 19:6131-6143. [PMID: 24115809 PMCID: PMC3787342 DOI: 10.3748/wjg.v19.i37.6131] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2013] [Revised: 08/13/2013] [Accepted: 08/20/2013] [Indexed: 02/06/2023] Open
Abstract
Acute variceal hemorrhage, a life-threatening condition that requires a multidisciplinary approach for effective therapy, is defined as visible bleeding from an esophageal or gastric varix at the time of endoscopy, the presence of large esophageal varices with recent stigmata of bleeding, or fresh blood visible in the stomach with no other source of bleeding identified. Transfusion of blood products, pharmacological treatments and early endoscopic therapy are often effective; however, if primary hemostasis cannot be obtained or if uncontrollable early rebleeding occurs, transjugular intrahepatic portosystemic shunt (TIPS) is recommended as rescue treatment. The TIPS represents a major advance in the treatment of complications of portal hypertension. Acute variceal hemorrhage that is poorly controlled with endoscopic therapy is generally well controlled with TIPS, which has a 90% to 100% success rate. However, TIPS is associated with a mortality of 30% to 50% in such a setting. Emergency TIPS should be considered early in patients with refractory variceal bleeding once medical treatment and endoscopic sclerotherapy failure, before the clinical condition worsens. Furthermore, admission to specialized centers is mandatory in such a setting and regional protocols are essential to be organized effectively. This review article discusses initial management and then focuses on the specific role of TIPS as a primary therapy to control acute variceal hemorrhage, particularly as a rescue therapy following failure of endoscopic approaches.
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Dignan FL, Wynn RF, Hadzic N, Karani J, Quaglia A, Pagliuca A, Veys P, Potter MN. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. Br J Haematol 2013; 163:444-57. [PMID: 24102514 DOI: 10.1111/bjh.12558] [Citation(s) in RCA: 215] [Impact Index Per Article: 17.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
DIAGNOSIS It is recommended that the diagnosis of veno-occlusive disease (sinusoidal obstruction syndrome) [VOD (SOS)] be based primarily on established clinical criteria (modified Seattle or Baltimore criteria) (1A). Ultrasound imaging may be helpful in the exclusion of other disorders in patients with suspected VOD (SOS) (1C). It is recommended that liver biopsy be reserved for patients in whom the diagnosis of VOD (SOS) is unclear and there is a need to exclude other diagnoses (1C). It is recommended that liver biopsies are undertaken using the transjugular approach in order to reduce the risks associated with the procedure (1C). It is suggested that the role of plasminogen activator inhibitor 1 levels remains an area for further research but that these levels should not form part of the routine diagnostic work-up for VOD (SOS) at present (2C). RISK FACTORS It is recommended that patients are assessed for risk factors for VOD (SOS) and that these risk factors are addressed prior to haematopoietic stem cell transplantation (1A). PROPHYLAXIS Defibrotide is recommended at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in children undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (1A). Defibrotide is suggested at a dose of 6.25 mg/kg intravenously four times daily for the prevention of VOD (SOS) in adults undergoing allogeneic stem cell transplantation with the following risk factors: pre-existing hepatic disease, second myeloablative transplant, allogeneic transplant for leukaemia beyond second relapse, conditioning with busulfan-containing regimens, prior treatment with gemtuzumab ozogamicin, diagnosis of primary haemophagocytic lymphohistiocytosis, adrenoleucodystrophy or osteopetrosis (2B). Prostaglandin E1 is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy and toxicity (1B). Pentoxifylline is not recommended in the prophylaxis of VOD (SOS) due to lack of efficacy (1A). Ursodeoxycholic acid is suggested for use in the prophylaxis of VOD (SOS) (2C). Heparin (unfractionated and low molecular weight) is not suggested for use in the prophylaxis of VOD (SOS) due to the risk of increased toxicity (2B). Antithrombin is not suggested for the prophylaxis of VOD (SOS) due to lack of efficacy (2B). TREATMENT Defibrotide is recommended in the treatment of VOD (SOS) in adults and children (1B). Tissue plasminogen activator is not recommended for use in the treatment of VOD (SOS) due to the associated risk of haemorrhage (1B). N-acetylcysteine is not routinely recommended for use in the treatment of veno-occlusive disease due to lack of efficacy (1A). Methylprednisolone may be considered for use in the treatment of veno-occlusive disease with the appropriate caveats of caution regarding infection (2C). Judicious clinical care, particularly in the management of fluid balance, is recommended in the management of VOD (SOS) (1C). Early discussion with critical care specialists and a specialist hepatology unit is recommended in the management of VOD (SOS) and other treatment options including transjugular intrahepatic portosystemic shunt or hepatic transplantation may be considered (1C). SUMMARY A joint working group established by the Haemato-oncology subgroup of the British Committee for Standards in Haematology (BCSH) and the British Society for Blood and Marrow Transplantation (BSBMT) has reviewed the available literature and made recommendations for the diagnosis and management of veno-occlusive disease of the liver following haematopoietic stem cell transplantation (HSCT). This guideline includes recommendations for both prophylaxis and treatment of the condition and includes recommendations for children and adults undergoing HSCT.
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Affiliation(s)
- Fiona L Dignan
- Department of Haematology, Central Manchester NHS Foundation Trust, Manchester, UK
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36
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Abstract
OBJECTIVE The purpose of this article is to review the indications, outcomes, complications, patient selection, and technical aspects of creating a transjugular intrahepatic portosystemic shunt (TIPS). CONCLUSION The best available evidence supports the use of TIPS in secondary prevention of variceal bleeding and in refractory ascites, although TIPS is also commonly used for other indications such as Budd-Chiari syndrome, hepatic hydrothorax, and acute variceal hemorrhage. The TIPS procedure was revolutionized by the introduction of covered stents, which dramatically improved long-term shunt patency.
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37
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Abstract
Primary damage to hepatic vessels is rare. (i) Hepatic arterial disorders, related mostly to iatrogenic injury and occasionally to systemic diseases, lead to ischemic cholangiopathy. (ii) Hepatic vein or inferior vena cava thrombosis, causing primary Budd-Chiari syndrome, is related typically to a combination of underlying prothrombotic conditions, particularly myeloproliferative neoplasms, factor V Leiden, and oral contraceptive use. The outcome of Budd-Chiari syndrome has markedly improved with anticoagulation therapy and, when needed, angioplasty, stenting, TIPS, or liver transplantation. (iii) Extrahepatic portal vein thrombosis is related to local causes (advanced cirrhosis, surgery, malignant or inflammatory conditions), or general prothrombotic conditions (mostly myeloproliferative neoplasms or factor II gene mutation), often in combination. Anticoagulation at the early stage prevents thrombus extension and, in 40% of the cases, allows for recanalization. At the late stage, gastrointestinal bleeding related to portal hypertension can be prevented in the same way as in cirrhosis. (iv) Sinusoidal obstruction syndrome (or venoocclusive disease), caused by agents toxic to bone marrow progenitors and to sinusoidal endothelial cells, induces portal hypertension and liver dysfunction. Decreasing the intensity of myeloablative regimens reduces the incidence of sinusoidal toxicity. (v) Obstruction of intrahepatic portal veins (obliterative portal venopathy) can be associated with autoimmune diseases, prothrombotic conditions, or HIV infection. The disease can eventually be complicated with end-stage liver disease. Extrahepatic portal vein obstruction is common. Anticoagulation should be considered. (vi) Nodular regenerative hyperplasia is induced by the uneven perfusion due to obstructed sinusoids, or portal or hepatic venules. It causes pure portal hypertension.
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Affiliation(s)
- Aurélie Plessier
- Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France
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Peng Q, He DZ, Li JS. Clinical research of hepatic veno-occlusive disease: current status and future prospects. Shijie Huaren Xiaohua Zazhi 2012; 20:1030-1035. [DOI: 10.11569/wcjd.v20.i12.1030] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Hepatic veno-occlusive disease (HVOD) is one of the main complications of hematopoietic stem cell transplantation (HSCT). Its pathogenesis is mainly associated with a local hypercoagulable state, and the main pathological changes are occlusion of terminal hepatic venules and necrosis of liver cells. The diagnosis of HVOD depends on a liver biopsy. Identifying and avoiding the risk factors are main measures to reduce the incidence and mortality of HVOD, since drug prophylaxis lacks exact effect and has significant adverse reactions. Defibrotide is the most effective therapy for HVOD, while the efficacy of other drugs still needs to be verified. In this paper, we will review the current status and future prospects of clinical research of HVOD.
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Levitsky J, Walzer N. Hepatobiliary Complications of Hematopoietic Cell Transplantation. SCHIFF'S DISEASES OF THE LIVER 2011:255-270. [DOI: 10.1002/9781119950509.ch10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Bhogal HK, Sanyal AJ. Using transjugular intrahepatic portosystemic shunts for complications of cirrhosis. Clin Gastroenterol Hepatol 2011; 9:936-46; quiz e123. [PMID: 21699820 PMCID: PMC3200495 DOI: 10.1016/j.cgh.2011.06.013] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2011] [Revised: 06/01/2011] [Accepted: 06/05/2011] [Indexed: 02/07/2023]
Abstract
Transjugular intrahepatic portosystemic shunt (TIPS) decompresses the portal venous system. TIPS has been used to manage the complications of portal hypertension in cirrhosis, including variceal hemorrhage and refractory ascites. The uncoated TIPS stents are limited by stent stenosis; however, the introduction of coated stents has decreased this. With the introduction of coated stents, we must reevaluate the utility of TIPS in the management of complications of portal hypertension.
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Mahgerefteh SY, Sosna J, Bogot N, Shapira MY, Pappo O, Bloom AI. Radiologic Imaging and Intervention for Gastrointestinal and Hepatic Complications of Hematopoietic Stem Cell Transplantation. Radiology 2011; 258:660-71. [DOI: 10.1148/radiol.10100025] [Citation(s) in RCA: 74] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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Abstract
This guideline has been approved by the American Association for the Study of Liver Diseases (AASLD) and represents the position of the association.
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Affiliation(s)
- Laurie D DeLeve
- Division of Gastrointestinal and Liver Diseases and the Research Center for Liver Diseases, University of Southern California Keck School of Medicine, Los Angeles, CA, USA
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Membreno FE, Ortiz J, Foster PF, Wright F, Ostrower VS, Metter JD, Palma-Vargas J, LeMaistre CF. Liver transplantation for sinusoidal obstructive syndrome (veno-occlusive disease): case report with review of the literature and the UNOS database. Clin Transplant 2008; 22:397-404. [PMID: 18430045 DOI: 10.1111/j.1399-0012.2008.00814.x] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
BACKGROUND Severe sinusoidal obstructive syndrome (SOS) is a life-threatening complication of stem cell transplantation. We report the case of a young man transplanted for SOS. METHOD A single chart review with query of the United Network of Organ Sharing database and review of the medical literature. CASE A 23-yr-old male diagnosed with chronic myeloid leukemia underwent a matched unrelated stem cell transplant. The conditioning regimen included high-dose cyclophosphamide and busulfan. Within one month, he developed painful hepatomegaly, jaundice, ascites, and weight gain, and was diagnosed with biopsy-proven SOS. Despite therapy with defibrotide, he continued to deteriorate with the development of progressive renal failure and encephalopathy. The patient underwent orthotopic liver transplantation. After surgery, he developed cytomegalovirus infection and six wk later presented with a bile leak, hepatic artery thrombosis, and a liver abscess. A repeat bone marrow biopsy showed no evidence of recurrent disease. Although the patient was listed for re-transplantation, he succumbed prior to an organ becoming available. CONCLUSION Severe SOS in the setting of bone marrow transplantation portends a poor prognosis. Careful patient selection, timing, and perhaps less immunosuppression should be considered when performing a liver transplantation in the setting of severe SOS.
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Affiliation(s)
- Fernando E Membreno
- Texas Transplant Institute, Methodist Specialty and Transplant Hospital, San Antonio, TX 78229, USA.
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Del Toro G. Defibrotide in sinusoidal obstruction syndrome: mounting evidence in pediatric patients (Commentary on Qureshi et al., page 831). Pediatr Blood Cancer 2008; 50:735-6. [PMID: 18286500 DOI: 10.1002/pbc.21470] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/10/2022]
Affiliation(s)
- Gustavo Del Toro
- Jack Martin Division of Pediatric Hematology/Oncology, Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1208, New York, New York 10029, USA.
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45
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Hepatic veno-occlusive disease followed by esophageal varix rupture after hematopoietic stem cell transplantation in a 4-year-old boy with stage 4 neuroblastoma. J Pediatr Hematol Oncol 2008; 30:63-5. [PMID: 18176184 DOI: 10.1097/mph.0b013e318159a510] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Hepatic veno-occlusive disease (HVOD) is one of the major potential complications associated with high-dose chemotherapy with stem cell transplantation in children. However, esophageal varix rupture after HVOD has, to our knowledge, never been reported before. Here we report a case of a 4-year-old boy with stage 4 neuroblastoma with potentially fatal HVOD followed by esophageal varix rupture and massive intestinal bleeding after high-dose chemotherapy with autologous peripheral blood stem cell transplantation. We successfully treated him by endoscopic variceal ligation and administration of somatostatin analog.
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Abstract
Our goal is to provide a detailed review of veno-occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.
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Affiliation(s)
- Ulas-Darda Bayraktar
- Department of Internal Medicine, Interfaith Medical Center, 229 Parkville Ave Apt# 4B, Brooklyn, NY 11230, United States.
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47
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Abstract
This review will discuss the use of transjugular intrahepatic portosystemic stent-shunt in a number of relatively uncommon clinical situations. In particular, we will focus our paper on the use of transjugular intrahepatic portosystemic stent-shunt for hepatic hydrothorax, hepatopulmonary syndrome, veno-occlusive disease, portal hypertensive gastropathy and gastric antral vascular ectasia, before surgery and after liver transplantation.
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Affiliation(s)
- George Therapondos
- Division of Gastroenterology, Department of Medicine, University of Toronto, Toronto General Hospital, Toronto, Ontario, Canada.
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48
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Senzolo M, Patch D, Cholongitas E, Triantos C, Marelli L, Stigliano R, Dhillon A, Burroughs A. Severe venoocclusive disease after liver transplantation treated with transjugular intrahepatic portosystemic shunt. Transplantation 2006; 82:132-5. [PMID: 16861953 DOI: 10.1097/01.tp.0000225799.76828.ce] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Venoocclusive disease (VOD) is due to hepatic sinusoidal lining injury leading to portal hypertension; its incidence after liver transplantation is about 2%. When severe, it does not respond to medical therapy and has a high mortality; retransplantation is the only therapeutic option. However, there are no detailed data regarding the use of transjugular intrahepatic portosystemic shunt for VOD after liver transplantation. We describe two patients who developed severe VOD after liver transplantation, failed defibrotide therapy, and were treated by transjugular intrahepatic portosystemic shunt (TIPS). The portal hypertension resolved completely and one had full histological recovery. We believe that TIPS should be attempted as it may resolve progressive portal hypertension and the hepatic congestion, while allowing the clinician time for listing for further liver transplantation if the patient fails to respond.
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Affiliation(s)
- Marco Senzolo
- Liver Transplantation and Hepatobiliary Unit, Royal Free Hospital, London, United Kingdom
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49
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Abstract
Hepatic sinusoidal obstruction syndrome is frequently linked to high-dose chemotherapy/total-body irradiation in recipients of haematopoietic stem cell transplantation, long-term use of azathioprine after organ transplantation and other chemotherapeutic agents. The incidence of hepatic sinusoidal obstruction syndrome varies from 0% to 70%, and is decreasing. Disease risk is higher in patients with malignancies, hepatitis C virus infection, those who present late, when norethisterone is used to prevent menstruation, and when broad-spectrum antibiotics and antifungals are used during and after the conditioning therapy. Hepatic sinusoidal obstruction syndrome presents with tender hepatomegaly, hyperbilirubinaemia and ascites, and diagnosis is mainly clinical (Seattle and Baltimore Criteria). Imaging excludes biliary obstruction and malignancy, but cannot establish accurate diagnosis. Hepatic sinusoidal obstruction syndrome may be prevented by avoiding the highest risk regimens, using non-myelo-ablative regimens, and reducing total-body irradiation dose. Treatment is largely symptomatic and supportive, because 70-80% of patients recover spontaneously. Tissue plasminogen activator plus heparin improves outcome in <30% of cases. Defibrotide, a polydeoxyribonucleotide, is showing encouraging results. Transjugular intrahepatic porto-systemic shunt relieves ascites, but does not improve outcome. Liver transplantation may be an option in the absence of malignancy. Prognosis is variable and depends on disease severity, aetiology and associated conditions. Death is most commonly caused by renal or cardiopulmonary failure.
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Affiliation(s)
- A Helmy
- Department of Liver Transplantation, Hepatobiliary and Pancreatic Surgery, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
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50
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Boyer TD, Haskal ZJ. American Association for the Study of Liver Diseases Practice Guidelines: the role of transjugular intrahepatic portosystemic shunt creation in the management of portal hypertension. J Vasc Interv Radiol 2005; 16:615-29. [PMID: 15872315 DOI: 10.1097/01.rvi.0000157297.91510.21] [Citation(s) in RCA: 96] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Affiliation(s)
- Thomas D Boyer
- Liver Research Institute, University of Arizona School of Medicine, AHSC 245136, Tucson, 85750, USA.
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