Autoimmune hemolytic anemia

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Abstract

Red blood cell (RBC) autoantibodies are a relatively uncommon cause of anemia. However, autoimmune hemolytic anemia (AIHA) must be considered in the differential diagnosis of hemolytic anemias, especially if the patient has a concomitant lymphoproliferative disorder, autoimmune disease, or viral or mycoplasmal infection. Classifications of AIHA include warm AIHA, cold agglutinin syndrome, paroxysmal cold hemoglobinuria, mixed-type AIHA, and drug-induced AIHA. Characteristics of the autoantibodies are responsible for the various clinical entities. As a result, diagnosis is based on the clinical presentation and a serologic work-up. For each classification of AIHA, this review discusses the demographics, etiology, clinical presentation, laboratory evaluation, and treatment options. Copyright 2002 Wiley-Liss, Inc.

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J Ravetch, J Kinet (1991)

Recent advances in the structural analysis of the genes and proteins for immunoglobulin Fc domain receptors have provided a molecular characterization of this complex family. The wide cellular distribution of these receptors and their functional heterogeneity are reflected in the diversity of molecules which bind antibody and immune complexes. The detailed analysis of the IgG and IgE Fc receptors has indicated that these molecules have evolved from a common precursor through gene duplication. Similarities among these receptors, in both structure and function have emerged. Thus, the Fc receptors provide an example of a class of molecules in which conserved domains are combined with divergent sequences to yield a diversity of function.

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Autoimmune haemolysis: an 18-year study of 865 cases referred to a regional transfusion centre.

R Sokol, S Hewitt, B K Stamps (1981)

Clinical and serological records of 865 patients with confirmed autoimmune haemolysis (AIH)--a much larger series than any previously reported--were critically reviewed nd analysed. A proposed new classification for AIH based on serological findings differs from traditional classifications in that a new category of "mixed" AIH has been defined in which both "warm" and "cold" autoantibodies are present, and both are capable of causing haemolysis. Patients in this mixed group tend to have severe disease that may run a chronic intermittent course. The presentation of cold agglutinin disease is much more variable than has been seen in previous studies, haemolysis due to low titre autoantibodies being common. The AIH associated with pregnancy, usually considered as being of bad prognosis, is often mild and self limiting in the absence of other associated disorders.

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Efficacy of intravenous immunoglobulin in the treatment of autoimmune hemolytic anemia: results in 73 patients.

C Cummingham-Rundles, John D G Flores, Stephen Newland … (1993)

To determine whether warm-antibody autoimmune hemolytic anemia (AIHA) responds to treatment with intravenous gammaglobulin (IVGG), we conducted separate pilot studies at three institutions enrolling a total of 37 patients. We combined these results with a review of 36 cases of AIHA treated with IVGG reported in the literature. Sixteen clinical variables were examined to determine associations with response to IVGG. Overall, 29 of 73 patients (39.7%) responded to IVGG therapy. Two variables were strongly related to a good response to IVIG: the presence of hepatomegaly (with and without splenomegaly) and a low pre-treatment hemoglobin. A trend towards a better response was observed in the 11 children. Overall, IVGG provided acute benefit in only 1/3 of patients and therefore cannot be recommended as standard therapy for AIHA. It may, however, be useful as adjunctive treatment in selected cases, such as in those with a pre-treatment hemoglobin < 6-7 gm/dl or those with hepatomegaly, and in clinical settings where the toxicity of other treatments may be an important consideration.