Xu XT, Wang BH, Wang Q, Guo YJ, Zhang YN, Chen XL, Fang YF, Wang K, Guo WH, Wen ZZ. Idiopathic hypereosinophilic syndrome with hepatic sinusoidal obstruction syndrome: A case report and literature review. World J Gastrointest Surg 2023; 15(7): 1532-1541 [PMID: 37555104 DOI: 10.4240/wjgs.v15.i7.1532]
Corresponding Author of This Article
Zhen-Zhen Wen, PhD, Associate Chief Physician, Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, No. 3 East Qingchun Road, Hangzhou 310016, Zhejiang Province, China. 3315019@zju.edu.cn
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Xu-Tao Xu, Bing-Hong Wang, Yang-Jie Guo, Yu-Ning Zhang, Xiao-Li Chen, Yan-Fei Fang, Kan Wang, Zhen-Zhen Wen, Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, Hangzhou 310016, Zhejiang Province, China
Qiang Wang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
Wen-Hao Guo, Department of Pathology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, Hangzhou 310016, Zhejiang Province, China
Author contributions: Xu XT and Wang BH contributed to manuscript writing and editing; Wang Q, Guo YJ and Zhang YN were responsible for data collection; Chen XL, Fang YF and Wang K reviewed the literature; Guo WH contributed to pathological diagnosis; Wen ZZ contributed to conceptualization and supervision; All authors have read and approved the final manuscript to be submitted.
Supported bythe National Science of Foundation Committee of Zhejiang Province, No. LY22H160003; and the Zhejiang Provincial Medical and Health Science Foundation; No. 2021441200 and No. 2021RC083.
Informed consent statement: Written informed consent was obtained from the patient for treatment and publication of this case report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Zhen-Zhen Wen, PhD, Associate Chief Physician, Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, No. 3 East Qingchun Road, Hangzhou 310016, Zhejiang Province, China. 3315019@zju.edu.cn
Received: March 8, 2023 Peer-review started: March 8, 2023 First decision: April 2, 2023 Revised: April 17, 2023 Accepted: May 17, 2023 Article in press: May 17, 2023 Published online: July 27, 2023 Processing time: 135 Days and 12.3 Hours
Abstract
BACKGROUND
Hypereosinophilic syndrome (HES) is classified as primary, secondary or idiopathic. Idiopathic HES (IHES) has a variable clinical presentation and may involve multiple organs causing severe damage. Hepatic sinusoidal obstruction syndrome (HSOS) is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules, with occlusion of the hepatic venules, and hepatocyte necrosis. We report a case of IHES with HSOS of uncertain etiology.
CASE SUMMARY
A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for > 5 mo. He had previously undergone antiallergic treatment and herbal therapy in the local hospital, but the symptoms recurred. Relevant examinations were completed after admission. Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils (23%) with approximately normal morphology. Ultrasound-guided hepatic aspiration biopsy indicated HSOS. Contrast-enhanced computed tomography (CT) of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites. The patient was initially diagnosed with IHES and hepatic venule occlusion. Prednisone, low molecular weight heparin and ursodeoxycholic acid were given for treatment, followed by discontinuation of low molecular weight heparin due to ecchymosis. Routine blood tests, biochemical tests, and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.
CONCLUSION
Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.
Core Tip: Idiopathic hypereosinophilic syndrome (IHES) is characterized by a continuous increase and abnormal accumulation of eosinophils in the peripheral blood. Its clinical manifestations vary, and may involve multiple organs and cause serious damage. Hepatic sinusoidal obstruction syndrome (HSOS) can lead to veno-occlusion and hepatocyte necrosis. We report a case of IHES with HSOS. However, the cause of HSOS was unknown, and we could not determine whether it was caused by herbal medicine or IHES. Prednisone, low molecular weight heparin and ursodeoxycholic acid were given. Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.
