Hematologic Diseases.

Autoimmune destruction of circulating blood cells in autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) is often seen in autoimmune diseases and lymhoid malignancies. Erythrocytes or platelets that are recognized by autoantibodies are rapidly phagocytosed by macrophages. Although much is known about the mechanisms behind macrophage-mediated destruction of sensitized blood cells, less is known about the genetics behind AIHA and ITP. We here review what is known about the ethiology of AIHA and ITP, with particular emphasis on the role of genetic factors behind autoantibody production, T cell activation and apoptosis, and Fcγ receptor polymorphisms. The importance of inhibitory regulation of macrophages through CD47/SIRPα interaction, and its significance for autoimmune hematological disease is also discussed. [ABSTRACT FROM AUTHOR]

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