Authors: Benesch, Martin; Urban, Christian; Platzbecker, Uwe; Passweg, Jakob

Source: Expert Review of Clinical Immunology, Volume 5, Number 3, May 2009 , pp. 341-348(8)

Publisher: Expert Reviews

Abstract:

Evans syndrome (ES) is a chronic hematological disorder characterized by autoimmune hemolytic anemia and immune-mediated thrombocytopenia that often requires profound and long-term immunosuppression. Only a few small case series or single case studies of autologous and allogeneic hematopoietic stem cell transplantation (HSCT) in patients with ES have been reported in the literature, with long-term remissions being observed after both autologous and allogeneic HSCT. Patients with ES suffering from refractory disease, multiple relapses and serious disease-related complications should be offered allogeneic HSCT, which is the only treatment with curative potential. Autologous HSCT might be preferable in patients with serious pre-existing comorbidities lacking an HLA-identical donor. Owing to the rarity of this disease and the small number of patients receiving HSCT for ES, prospective controlled studies on this approach are not available. A prospective registration of patients transplanted for ES would allow the development of optimal transplant strategies.

Keywords: allogeneic hematopoietic stem cell transplantation; autologous hematopoietic stem cell transplantation; Evans syndrome

Document Type: Research article

DOI: http://dx.doi.org/10.1586/eci.09.9

Affiliations: 1: Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 30, A-8036 Graz, Austria., Email: martin.benesch@klinikum-graz.at

Publication date: 2009-05-01

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• Expert Review of Clinical Immunology provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in disease states with a strong immunologic component, such as allergic asthma, rheumatoid arthritis, inflammatory bowel disease, psoriasis and multiple sclerosis
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