Current treatment strategies in autoimmune hemolytic disorders

Expert Review of Hematology
Wilma Barcellini

Abstract

Autoimmune hemolytic anemia (AIHA) is a heterogeneous disease usually classified according to the thermal range of the autoantibody in warm, cold and mixed forms. The treatment of AIHA is still not evidence-based. Corticosteroids are the first-line therapy for warm AIHA. For refractory/relapsed cases, the choice is between splenectomy (effective in ∼70% cases but with a presumed cure rate of 20%) and rituximab (effective in ∼70-80% of cases), which is becoming the preferred second-line treatment, and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins and danazol. For severe or refractory cases, last option treatments are plasma-exchange, high-dose cyclophosphamide and alemtuzumab. As regards cold agglutinin disease, rituximab is now recommended as first-line treatment.

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Citations

Feb 13, 2016·Expert Review of Hematology·Wilma Barcellini
Sep 12, 2018·Expert Review of Clinical Immunology·Wilma BarcelliniAnna Zaninoni
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May 26, 2021·Journal of Clinical Apheresis·Petra RichterRené Dörfelt

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Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person's own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in the circulation. Discover the latest research on AIHA here.

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