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Benesch M, Urban C, Platzbecker U, et al. Stem cell transplantation for patients with Evans syndrome. Expert Rev Clin Immunol. 2009;5(3):341-8doi: 10.1586/eci.09.9.
Benesch, M., Urban, C., Platzbecker, U., & Passweg, J. (2009). Stem cell transplantation for patients with Evans syndrome. Expert review of clinical immunology, 5(3), 341-8. https://doi.org/10.1586/eci.09.9
Benesch, Martin, et al. "Stem cell transplantation for patients with Evans syndrome." Expert review of clinical immunology vol. 5,3 (2009): 341-8. doi: https://doi.org/10.1586/eci.09.9
Benesch M, Urban C, Platzbecker U, Passweg J. Stem cell transplantation for patients with Evans syndrome. Expert Rev Clin Immunol. 2009 May;5(3):341-8. doi: 10.1586/eci.09.9. PMID: 20477011.
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Expert Rev Clin Immunol. 2009 May;5(3):341-8. doi: 10.1586/eci.09.9.

Stem cell transplantation for patients with Evans syndrome.

Expert review of clinical immunology

Martin Benesch, Christian Urban, Uwe Platzbecker, Jakob Passweg

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 protected]

PMID: 20477011 DOI: 10.1586/eci.09.9

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.

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