Department of Haematology, Leeds Teaching Hospitals, Leeds, United Kingdom.
Hematology Am Soc Hematol Educ Program. 2018 Nov 30;2018(1):382-389. doi: 10.1182/asheducation-2018.1.382.
The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test. Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA. Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease (CAD), either as monotherapy or combined with bendamustine. Complement inhibitors have shown utility in stabilizing AIHA patients with acute severe hemolysis. Future prospects are discussed and include the C1s inhibitor BIVV009 (sutimlimab) that is now entering phase 3 studies for CAD.
自身免疫性溶血性贫血 (AIHA) 的诊断可以采用逐步的方法，旨在确定实验室和临床溶血的证据，然后用直接抗球蛋白试验确定溶血的免疫性质。一旦排除了这些发现的其他原因，就可以确定为 AIHA，临床医生必须寻找继发原因，并确定 AIHA 的类型。利妥昔单抗现在是原发性温抗体型 AIHA 的二线首选治疗药物，也是原发性冷凝集素病 (CAD) 的一线治疗药物，无论是单药治疗还是与苯达莫司汀联合治疗。补体抑制剂在稳定急性严重溶血的 AIHA 患者方面显示出了效用。未来的前景包括 C1s 抑制剂 BIVV009（sutimlimab），目前正在 CAD 的 3 期研究中。
