Autoimmune hemolytic anemia (AIHA) is an acquired, heterogeneous group of diseases which includes warm AIHA, cold agglutinin disease (CAD), mixed AIHA, paroxysmal cold hemoglobinuria and atypical AIHA. Currently CAD is defined as a chronic, clonal lymphoproliferative disorder, while the presence of cold agglutinins underlying other diseases is known as cold agglutinin syndrome. AIHA is mediated by autoantibodies directed against red blood cells (RBCs) causing premature erythrocyte destruction. The pathogenesis of AIHA is complex and still not fully understood. Recent studies indicate the involvement of T and B cell dysregulation, reduced CD4+ and CD25+ Tregs, increased clonal expansions of CD8 + T cells, imbalance of Th17/Tregs and Tfh/Tfr, and impaired lymphocyte apoptosis. Changes in some RBC membrane structures, under the influence of mechanical stimuli or oxidative stress, may promote autohemolysis. The clinical presentation and treatment of AIHA are influenced by many factors, including the type of AIHA, degree of hemolysis, underlying diseases, presence of concomitant comorbidities, bone marrow compensatory abilities and the presence of fibrosis and dyserthropoiesis. The main treatment for AIHA is based on the inhibition of autoantibody production by mono- or combination therapy using GKS and/or rituximab and, rarely, immunosuppressive drugs or immunomodulators. Reduction of erythrocyte destruction via splenectomy is currently the third line of treatment for warm AIHA. Supportive treatment including vitamin supplementation, recombinant erythropoietin, thrombosis prophylaxis and the prevention and treatment of infections is essential. New groups of drugs that inhibit immune responses at various levels are being developed intensively, including inhibition of antibody-mediated RBCs phagocytosis, inhibition of B cell and plasma cell frequency and activity, inhibition of IgG recycling, immunomodulation of T lymphocytes function, and complement cascade inhibition. Recent studies have brought about changes in classification and progress in understanding the pathogenesis and treatment of AIHA, although there are still many issues to be resolved, particularly concerning the impact of age-associated changes to immunity.

中文翻译：

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自身免疫性溶血性贫血：当前知识和观点


自身免疫性溶血性贫血（AIHA）是一组获得性异质性疾病，包括温性AIHA、冷凝集素病（CAD）、混合性AIHA、阵发性冷性血红蛋白尿和非典型AIHA。目前，CAD 被定义为一种慢性克隆性淋巴增殖性疾病，而其他疾病中存在的冷凝集素则被称为冷凝集素综合征。 AIHA 由针对红细胞 (RBC) 的自身抗体介导，导致红细胞过早破坏。 AIHA的发病机制很复杂，目前尚未完全了解。最近的研究表明，T 细胞和 B 细胞失调、CD4+ 和 CD25+ Tregs 减少、CD8+ T 细胞克隆扩增增加、Th17/Tregs 和 Tfh/Tfr 失衡以及淋巴细胞凋亡受损。在机械刺激或氧化应激的影响下，一些红细胞膜结构的变化可能会促进自溶血。 AIHA的临床表现和治疗受多种因素影响，包括AIHA的类型、溶血程度、基础疾病、合并症、骨髓代偿能力以及纤维化和造血障碍的存在。 AIHA 的主要治疗方法是通过使用 GKS 和/或利妥昔单抗以及罕见的免疫抑制药物或免疫调节剂的单一或联合疗法来抑制自身抗体的产生。通过脾切除减少红细胞破坏是目前治疗温性 AIHA 的第三线。支持治疗包括补充维生素、重组促红细胞生成素、预防血栓形成以及预防和治疗感染是必不可少的。 抑制不同水平免疫反应的新药物组正在密集开发中，包括抑制抗体介导的红细胞吞噬作用、抑制B细胞和浆细胞频率和活性、抑制IgG再循环、T淋巴细胞功能的免疫调节和补体级联反应抑制。最近的研究改变了 AIHA 的分类，并在理解 AIHA 的发病机制和治疗方面取得了进展，尽管仍有许多问题有待解决，特别是与年龄相关的免疫变化的影响。