Current treatment strategies in autoimmune hemolytic disorders

Wilma Barcellini
Expert review of hematology · 2015 · vol. 8(5) , pp. 681–691 · doi:10.1586/17474086.2015.1073105 · PMID:26343892 · W1903281014
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Corticosteroids are first-line for warm AIHA, with rituximab and immunosuppressants used for refractory cases, while rituximab is now recommended first-line for cold agglutinin disease.

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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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MeSH descriptors

Anemia, Hemolytic, Autoimmune Adrenal Cortex Hormones Adrenal Cortex Hormones Adrenal Cortex Hormones Anemia, Hemolytic, Autoimmune Anemia, Hemolytic, Autoimmune Cyclophosphamide Cyclophosphamide Cyclophosphamide Humans Immunologic Factors Immunologic Factors Immunologic Factors Immunosuppressive Agents Immunosuppressive Agents Immunosuppressive Agents Rituximab Rituximab Rituximab Splenectomy

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