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温抗体型自身免疫性溶血性贫血

Warm autoimmune hemolytic anemia.

作者信息

Naik Rakhi

机构信息

Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, 1830 East Monument Street, Baltimore, MD 21230, USA.

出版信息

Hematol Oncol Clin North Am. 2015 Jun;29(3):445-53. doi: 10.1016/j.hoc.2015.01.001. Epub 2015 Mar 12.

DOI:10.1016/j.hoc.2015.01.001
PMID:26043384
Abstract

Warm autoimmune hemolytic anemia (AIHA) is defined as the destruction of circulating red blood cells (RBCs) in the setting of anti-RBC autoantibodies that optimally react at 37°C. The pathophysiology of disease involves phagocytosis of autoantibody-coated RBCs in the spleen and complement-mediated hemolysis. Thus far, treatment is aimed at decreasing autoantibody production with immunosuppression or reducing phagocytosis of affected cells in the spleen. The role of complement inhibitors in warm AIHA has not been explored. This article addresses the diagnosis, etiology, and treatment of warm AIHA and highlights the role of complement in disease pathology.

摘要

温抗体型自身免疫性溶血性贫血(AIHA)的定义是,在抗红细胞自身抗体存在的情况下,循环红细胞(RBC)被破坏,这些自身抗体在37°C时反应最佳。该疾病的病理生理学涉及脾脏中自身抗体包被的红细胞的吞噬作用以及补体介导的溶血。到目前为止,治疗旨在通过免疫抑制减少自身抗体的产生,或减少脾脏中受影响细胞的吞噬作用。补体抑制剂在温抗体型AIHA中的作用尚未得到探索。本文论述了温抗体型AIHA的诊断、病因和治疗,并强调了补体在疾病病理中的作用。

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