Subran Benjamin, Montagner Clémence, Lidove Olivier
Service de médecine interne, hôpital de la Croix Saint-Simon, Paris, France.
Rev Prat. 2020 Jan;70(1):79-87.
Autoimmune hemolytic anemias. Autoimmune hemolytic anemias (AIHA) are a rare cause of acquired hemolytic anemia, linked to the presence of an autoantibody directed against one or more antigens expressed on the surface of the red blood cell and certified by a positive direct antiglobulin test (DAT). AIHA can be non regenerative (10-20% of cases) and DAT may be negative (5% of cases). There are two main forms: warm antibodies AIHA (IgG positive TDA +/- C3d) and cold antibodies AIHA (C3d positive TDA), which differ in their underlying causes and treatment. Warm antibodies AIHA are in 50% of cases associated to B-cell chronic lymphoid leukemia, variable common immune deficiency, systemic lupus or drug; the treatment is based on short corticosteroid therapy (3 to 6 months) and rituximab is the 2nd line treatment. Cold antibodies AIHA are of two types: either post-infectious (mycoplasma, EBV), or linked to cold agglutinin disease that is indolent B clonal hemopathy; the treatment is primarily symptomatic and relies on cold protection measures. Corticosteroid therapy and splenectomy are ineffective. In cases of severe anemia, treatment with rituximab alone or in combination with chemotherapy is indicated.
自身免疫性溶血性贫血。自身免疫性溶血性贫血(AIHA)是获得性溶血性贫血的罕见病因,与针对红细胞表面表达的一种或多种抗原的自身抗体的存在有关,并通过直接抗球蛋白试验(DAT)阳性得以证实。AIHA可能是非再生性的(10% - 20%的病例),且DAT可能为阴性(5%的病例)。主要有两种形式:温抗体型AIHA(IgG阳性TDA +/- C3d)和冷抗体型AIHA(C3d阳性TDA),它们在潜在病因和治疗方面存在差异。温抗体型AIHA在50%的病例中与B细胞慢性淋巴细胞白血病、可变常见免疫缺陷、系统性红斑狼疮或药物有关;治疗基于短期皮质类固醇治疗(3至6个月),利妥昔单抗为二线治疗药物。冷抗体型AIHA有两种类型:一种是感染后型(支原体、EB病毒),另一种与冷凝集素病有关,后者是一种惰性B克隆性血液病;治疗主要是对症治疗,依靠保暖措施。皮质类固醇治疗和脾切除术无效。在严重贫血的病例中,可单独使用利妥昔单抗或联合化疗进行治疗。