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自身免疫性溶血性贫血的地理流行病学。

Geoepidemiology of autoimmune hemolytic anemia.

机构信息

Service of Hematology, University Hospitals of Canton Vaud (CHUV), Lausanne, Switzerland.

出版信息

Autoimmun Rev. 2010 Mar;9(5):A350-4. doi: 10.1016/j.autrev.2009.11.005. Epub 2009 Nov 27.

Abstract

Autoantibodies against red blood cell antigens are considered the diagnostic hallmark of AIHA: Direct antiglobulin test (DAT) completed by cytofluorometry and specific diagnostic monoclonal antibodies (mAbs) allow for a better understanding of autoimmune hemolytic anemia (AIHA) triggers. Once B-cell tolerance checkpoints are bypassed, the patient loses self-tolerance, if the AIHA is not also caused by an possible variety of secondary pathogenic events such as viral, neoplastic and underlying autoimmune entities, such as SLE or post-transplantation drawbacks; treatment of underlying diseases in secondary AIHA guides ways to curative AIHA treatment. The acute phase of AIHA, often lethal in former times, if readily diagnosed, must be treated using plasma exchange, extracorporeal immunoadsorption and/or RBC transfusion with donor RBCs devoid of the auto-antibody target antigen. Genotyping blood groups (www.bloodgen.com) and narrowing down the blood type subspecificities with diagnostic mAbs help to define the triggering autoantigen and to select well compatible donor RBC concentrates, which thus escape recognition by the autoantibodies.

摘要

自身抗体针对红细胞抗原被认为是自身免疫性溶血性贫血的诊断标志

直接抗球蛋白试验(DAT)通过细胞荧光术和特异性诊断单克隆抗体(mAbs)完成,有助于更好地了解自身免疫性溶血性贫血(AIHA)的触发因素。一旦 B 细胞耐受检查点被绕过,如果 AIHA 不是也由可能的多种继发性致病事件引起的,如病毒、肿瘤和潜在的自身免疫实体,如 SLE 或移植后缺陷;继发性 AIHA 中的基础疾病的治疗指导着治愈 AIHA 治疗的方法。AIHA 的急性期,如果以前易于诊断,往往是致命的,必须使用血浆置换、体外免疫吸附和/或输注无自身抗体靶抗原的供体 RBC 进行治疗。血型基因分型(www.bloodgen.com)和用诊断性 mAbs 缩小血型亚特异性有助于确定触发自身抗原,并选择相容性良好的供体 RBC 浓缩物,从而逃避自身抗体的识别。

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