Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review

Abdulgabar Salama, Abdulgabar Salama

Abstract

Until now, treatment of primary autoimmune hemolytic anemia of the warm type (wAIHA) is primarily based on immunosuppression. However, many patients do not respond adequately to treatment, and treated patients may develop severe side effects due to uncontrolled, mixed and/or long-lasting immunosuppression. Unfortunately, the newly used therapeutic monoclonal antibodies are unspecific and remain frequently ineffective. Thus, development of a specific therapy for AIHA is necessary. The ideal therapy would be the identification and elimination of the causative origin of autoimmunization and/or the correction or reprogramming of the dysregulated immune components. Blood transfusion is the most rapidly effective measure for patients who develop or may develop hypoxic anemia. Although some effort has been made to guide physicians on how to adequately treat patients with AIHA, a number of individual aspects should be considered prior to treatment. Based on my serological and clinical experience and the analysis of evidence-based studies, we remain far from any optimized therapeutic measures for all AIHA patients. Today, the old standard therapy using controlled steroid administration, with or without azathioprine or cyclophosphamide, is, when complemented with erythropoiesis-stimulating agents, still the most effective therapy in wAIHA. Rituximab or other monoclonal antibodies may be used instead of splenectomy in therapy-refractory patients.

Keywords: AIHA; Autoimmune hemolysis; Azathioprine; Blood transfusion; Corticosteroids; Cushing's syndrome; Cyclophosphamide; Erythropoietin; Intravenous IgG; Mycophenolate; Rituximab; Splenectomy.

Figures

Fig. 1
Fig. 1
How I treat patients with AIHA of warm type. Prior to treatment, all conditions including age, sex, comorbidity, and severity of hemolysis/anemia have to be considered.
Fig. 2
Fig. 2
An example of a typical case with refractory AIHA of warm type. Prior to treatment with darbepoetin patient's hemoglobin has never exceed 11.0 g/dl during oberservation. Cyclo = Cyclophosphamide, Pred = prednisolone, MMF = mycophenolate mofetil, Aza = azathioprine, Dexa = dexamethasone, ciclo = ciclosporin.

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