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[自身免疫性溶血性贫血]

[Autoimmune hemolytic anemia].

作者信息

Karasawa Masamitsu

机构信息

Blood Transfusion Service, University Hospital, Faculty of Medicine, Gunma University.

出版信息

Nihon Rinsho. 2008 Mar;66(3):520-3.

Abstract

Diagnosis of autoimmune hemolytic anemia (AIHA) requires both serologic evidence of an autoantibody and hemolysis. Based on the characteristic temperature reactivity of the autoantibody to red cell membranes, AIHA is classified into warm AIHA or cold AIHA (cold agglutinin disease and paroxysmal cold hemoglobinuria). Sensitized RBCs are destructed by intravascular and/or extravascular hemolysis. On the basis of etiology, AIHA are classified as idiopathic or secondary. The common cause of secondary AIHA is lymphoproliferative disorders, autoimmune diseases, and infections. The first line therapy of patients with warm AIHA is glucocorticoids and primary treatment for cold AIHA is avoiding cold exposure. The other standard treatments include splenectomy and immunosuppressive drugs. Recently, rituximab, a monoclonal anti-CD20 antibody, has been used in refractory AIHA with excellent responses.

摘要

自身免疫性溶血性贫血(AIHA)的诊断需要自身抗体的血清学证据和溶血表现。根据自身抗体对红细胞膜的特征性温度反应性,AIHA可分为温抗体型AIHA或冷抗体型AIHA(冷凝集素病和阵发性冷血红蛋白尿)。致敏红细胞通过血管内和/或血管外溶血被破坏。根据病因,AIHA可分为特发性或继发性。继发性AIHA的常见病因是淋巴增殖性疾病、自身免疫性疾病和感染。温抗体型AIHA患者的一线治疗是糖皮质激素,冷抗体型AIHA的主要治疗方法是避免寒冷暴露。其他标准治疗包括脾切除术和免疫抑制药物。近年来,利妥昔单抗,一种单克隆抗CD20抗体,已被用于治疗难治性AIHA并取得了良好疗效。

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