Current treatment strategies in autoimmune hemolytic disorders.
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.
a U.O. Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Wilma
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Barcellini
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000628:therapy*; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use; D013156:Splenectomy
D000305:Adrenal Cortex Hormones; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide
autoantibodies; autoimmune hemolytic anemia; cold agglutinin disease; therapeutic options; treatment