Treatment of Autoimmune Hemolytic Anemia

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The appropriate therapy of autoimmune hemolytic anemia (AIHA) is dependent on the correct diagnosis and classification of this family of hemolytic disorders. Although the majority of cases are warm AIHA, there are several distinct types of cold AIHA and a number of drug-induced etiologies of AIHA, which must be investigated to determine if stopping a drug will induce a remission. In warm AIHA, corticosteroids are standard, followed by consideration of splenectomy in recalcitrant cases. If steroids and splenectomy are insufficient, other forms of immunosuppressive therapy are typically initiated. In cold AIHA, keeping the patient warm in often sufficient, but therapy directed at an underlying lympholiferative disorder may be helpful. Brisk hemolysis, inadequate responses to therapy, and worsening anemia require transfusion therapy. Although the pretransfusion workup is made difficult by the presence of the autoantibody, transfusion services can usually provide blood safe for transfusion by excluding underlying alloantibodies. When transfusion is urgently required and compatible blood cannot be located, incompatible blood may be provided as a life-saving measure. Communication between the transfusion service and the hematologist is critical to assess the risks in these settings. Hemoglobin-based oxygen carriers may provide an important bridging therapy in the future. Requests for “least incompatible” blood do not enhance transfusion safety and often result in unnecessary delays.

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Corticosteroids

For warm AIHA, corticosteroids are the initial therapy of choice and the mainstay of therapy (Table 2). Many hematologists use a standard approach in adults, consisting of an initial course of prednisone, 60 to 100 mg per day for 1 to 3 weeks. There should be some evidence of clinical improvement within several days to 1 week. Approximately 80% of patients have a good initial response to corticosteroid therapy.3 After stabilization of hematologic parameters or the elimination of the requirement

Serologic Difficulties in AIHA

Transfusion therapy can be complicated in AIHA due to serologic complexities (Table 3). Although AIHA may present with an ABO and/or Rh typing discrepancy, accurate blood group identification usually can be determined without specialized techniques. In an emergency or if ABO results are not clear, group O donor RBCs are employed. Since the panagglutinin in the patient’s serum typically reacts with all donor RBCs, cross-matching blood may be a difficult and time-consuming process.

Patients with

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