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Shrestha AB, Yadav A, Sharma S, Sapkota UH, Farho MA, Islam MM, Abu Sayem MI, Nuruzzaman M. Parallel pathogens: Coexistence of chickenpox and idiopathic thrombocytopenic purpura-A case report. Clin Case Rep 2023; 11:CCR38014. [PMID: 37799566 PMCID: PMC10547856 DOI: 10.1002/ccr3.8014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2023] [Revised: 09/21/2023] [Accepted: 09/23/2023] [Indexed: 10/07/2023] Open
Abstract
This case report documents the unusual co-occurrence of immune thrombocytopena (ITP) and chickenpox in a 15-year-old girl. Initial symptoms included shortness of breath, chest pain, and heavy menstrual bleeding. Laboratory results revealed significant anemia and thrombocytopenia. Treatment involved blood transfusions, prednisolone, and iron supplementation. The patient's vesicular skin rash emerged 8 weeks later, prompting the combined diagnosis of ITP and chickenpox. Antiviral treatments, blood transfusions, and supportive care were used in the course of treatment, leading to full recovery. This case emphasizes the importance of prompt diagnosis, appropriate management, and regular follow-up for patients with both chickenpox and ITP. The coexistence of chickenpox and ITP poses a clinical challenge due to the complex interaction between the viral infection and the immune system. The exact mechanism linking these two conditions remains unclear, making it a baffling case that warrants investigation and further understanding. As low is the occurrence of hemorrhagic chickenpox, the presentation of simultaneous chicken pox with or following ITP was found to be rarer, and thus is this enigmatic case presented. Healthcare providers should remain vigilant about such co-occurrences to prevent complications. In order to improve treatment for instances with comparable clinical presentations and advance our collective knowledge, further study is required to better understand the mechanisms relating viral infections and ITP.
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Affiliation(s)
| | - Anuj Yadav
- M Abdur Rahim Medical College HospitalDinajpurBangladesh
| | | | | | | | | | | | - Md. Nuruzzaman
- Department of Internal MedicineM Abdur Rahim Medical College HospitalDinajpurBangladesh
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Muacevic A, Adler JR, Jacinto Correia C, Duro J, Aguiar P. Therapeutic Options in Refractory Evans Syndrome: A Case Report. Cureus 2022; 14:e32635. [PMID: 36654549 PMCID: PMC9842105 DOI: 10.7759/cureus.32635] [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: 12/17/2022] [Indexed: 12/23/2022] Open
Abstract
Evans syndrome is a rare autoimmune disease, characterized by at least two immune cytopenias, most frequently anemia and thrombocytopenia and rarely immune neutropenia. It has a variable clinical presentation and is rarely diagnosed in adults. It can be idiopathic or secondary to lymphoproliferative disease, infections, autoimmune diseases, drugs, and immunodeficiencies in about 50% of cases. It is characterized by a chronic, relapsing, potentially fatal course due to its hemorrhagic complications as well as complications associated with the long-term immunosuppressive treatment required to control the disease, such as infectious diseases, and cardiovascular and renal complications. Its prognosis depends on the underlying cause. Because of its rarity, the treatment is empirical, based mostly on case series and recommendations for the treatment of other immune cytopenias. The underlying disease and demographic characteristics also play an important role in choosing the treatment, which should be adapted individually to each patient. We present a case of an elderly patient with idiopathic autoimmune hemolytic anemia and thrombocytopenia, refractory to various treatment options.
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Kumar PA, Wazir A, Pu JJ. Resolution of Chronic Immune Thrombocytopenia Purpura after Autologous Hematopoietic Stem Cell Transplantation for Diffuse Large B-Cell Lymphoma. J Med Cases 2021; 12:37-40. [PMID: 33391580 PMCID: PMC7771832 DOI: 10.14740/jmc3607] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
Immune thrombocytopenic purpura (ITP) is a hematological disorder characterized by immune-mediated destruction of platelets that could be triggered by a number of causes. ITPs are usually treated with steroid, immunomodulators or immunosuppressors, and intravenous immunoglobulin therapy though refractory/relapsed status frequently occurs. It was suggested that autologous hematopoietic stem cell transplant (HSCT) after high-dose chemotherapy conditioning might improve ITP patients’ peripheral blood platelet counts via reorganizing disrupted immune balance in the hematopoietic and hematologic systems. In this case report, we describe how a patient, who suffered from both severe thrombocytopenia due to chronic ITP and refractory/relapsed diffuse large B-cell lymphoma (DLBCL), was managed to successfully receive autologous HSCT using carmustine, etoposide, cytarabine and melphalan (BEAM) conditioning regimens and how his chronic ITP was eventually cured after receiving autologous HSCT. This is the first clinical case in the world demonstrating that high-dose BEAM chemotherapy conditioned autologous HSCT could cure chronic ITP while successfully managing refractory/relapse DLBCL. The clinical hematology professionals and the patients will benefit from our experience in managing severe thrombocytopenia while conducting high-dose chemotherapy conditioning and autologous HSCT for DLBCL.
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Affiliation(s)
- Prashanth A Kumar
- Department of Medicine, State University of New York Upstate Medical University, Syracuse, NY, USA.,The authors equally contributed to this manuscript
| | - Ali Wazir
- Department of Medicine, State University of New York Upstate Medical University, Syracuse, NY, USA.,The authors equally contributed to this manuscript
| | - Jeffrey J Pu
- Department of Medicine, State University of New York Upstate Medical University, Syracuse, NY, USA
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Sulpizio ED, Raghunathan V, Shatzel JJ, Zilberman-Rudenko J, Worrest T, Sheppard BC, DeLoughery TG. Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study. Eur J Haematol 2019; 104:55-58. [PMID: 31594025 DOI: 10.1111/ejh.13336] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2019] [Revised: 10/01/2019] [Accepted: 10/04/2019] [Indexed: 12/01/2022]
Abstract
OBJECTIVE Evans syndrome, the combination of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) or autoimmune neutropenia, is associated with a high rate of relapsed/refractory disease. There are limited data on the efficacy of splenectomy for this condition. We reviewed patient outcomes after splenectomy for Evans syndrome compared to ITP at our institution. METHODS We performed a retrospective analysis of patients who underwent splenectomy for autoimmune cytopenias over a 23-year period with the intention of comparing disease relapse rates after splenectomy in patients with Evans syndrome and in those with ITP. RESULTS During the study period, 77 patients underwent splenectomy for ITP and seven underwent splenectomy for Evans syndrome. In the Evans cohort, splenectomy led to an 85.7% initial response rate with a 42.8% rate of relapse within one year and a long-term (one-year) response rate of 42.8%. In the ITP cohort, the initial response rate was 90.9% with a long-term response rate of 70.1%. CONCLUSION Our data suggest that long-term remission rates after splenectomy are lower in adults with Evans syndrome compared to those with ITP, although splenectomy may still be an acceptable treatment for certain patients with Evans syndrome. Our findings underscore the need for further research and development of additional therapeutic strategies for this patient population.
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Affiliation(s)
- Emilio D Sulpizio
- Department of Internal Medicine, Oregon Health and Science University, Portland, Oregon
| | - Vikram Raghunathan
- Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health and Science University, Portland, Oregon
| | - Joseph J Shatzel
- Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health and Science University, Portland, Oregon
| | | | - Tarin Worrest
- Department of Surgery, Oregon Health and Science University, Portland, Oregon
| | - Brett C Sheppard
- Department of Surgery, Oregon Health and Science University, Portland, Oregon
| | - Thomas G DeLoughery
- Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health and Science University, Portland, Oregon
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Műzes G, Sipos F. Issues and opportunities of stem cell therapy in autoimmune diseases. World J Stem Cells 2019; 11:212-221. [PMID: 31110602 PMCID: PMC6503459 DOI: 10.4252/wjsc.v11.i4.212] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2019] [Revised: 01/23/2019] [Accepted: 03/12/2019] [Indexed: 02/06/2023] Open
Abstract
The purpose of regenerative medicine is to restore or enhance the normal function of human cells, tissues, and organs. From a clinical point of view, the use of stem cells is more advantageous than differentiated cells because they can be collected more easily and in larger quantities, their proliferation capacity is more pronounced, they are more resistant in cell culture, their aging is delayed, they are able to form a number of cell lines, and they are able to promote vascularization of tissue carriers. The therapeutic use of stem cells for disease modification, immunomodulation, or regenerative purposes are undoubtedly encouraging, but most studies are still in their early stages, and the clinical results reported are not clear with regard to therapeutic efficacy and potential side effects. Uniform regulation of the clinical application of stem cells is also indispensable for this highly customizable, minimally invasive, individualized therapeutic method to become a successful and safe treatment alternative in many different autoimmune and autoinflammatory disorders.
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Affiliation(s)
- Györgyi Műzes
- Immunology Team, 2 Department of Internal Medicine, Semmelweis University, Szentkirályi Street 46, Budapest 1088, Hungary
| | - Ferenc Sipos
- Immunology Team, 2 Department of Internal Medicine, Semmelweis University, Szentkirályi Street 46, Budapest 1088, Hungary.
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Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med 2018; 9:171-184. [PMID: 30349415 PMCID: PMC6190623 DOI: 10.2147/jbm.s176144] [Citation(s) in RCA: 45] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%–73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person’s lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.
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Affiliation(s)
- José Carlos Jaime-Pérez
- Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,
| | - Patrizia Elva Aguilar-Calderón
- Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,
| | - Lorena Salazar-Cavazos
- Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,
| | - David Gómez-Almaguer
- Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,
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Abstract
Primary Evans syndrome (ES) is defined by the concurrent or sequential occurrence of immune thrombocytopenia and autoimmune hemolytic anemia in the absence of an underlying etiology. The syndrome is characterized by a chronic, relapsing, and potentially fatal course requiring long-term immunosuppressive therapy. Treatment of ES is hardly evidence-based. Corticosteroids are the mainstay of therapy. Rituximab has emerged as the most widely used second-line treatment, as it can safely achieve high response rates and postpone splenectomy. An increasing number of new genetic defects involving critical pathways of immune regulation identify specific disorders, which explain cases of ES previously reported as "idiopathic".
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