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.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
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