Kamesaki Toyomi
Center for Community Medicine, Jichi Medical University.
Rinsho Ketsueki. 2019;60(9):1100-1107. doi: 10.11406/rinketsu.60.1100.
In 2017, The British Society of Haematology published new guidelines for the diagnosis and management of autoimmune hemolytic anemia (AIHA). Usually this is diagnosed using a combination of clinical and laboratory findings of hemolysis using the direct antiglobulin test (DAT). The revised guidelines propose several steps to evaluate and diagnose patients with unexplained hemolysis and a negative DAT, and they recommend screening for cold agglutinin disease (CAD) using a direct agglutination test (DAggT) before evaluating the cold agglutinin titer. Rituximab is becoming the preferred second-line choice for steroid-refractory warm AIHA and the first-line choice for primary CAD. Rituximab is an off-label drug for use in AIHA treatment in Japan, but in future will be used as standard therapy. Anti-C1s antibody is a new drug for CAD that has entered phase 3 trials.
2017年，英国血液学学会发布了自身免疫性溶血性贫血（AIHA）诊断和管理的新指南。通常，这是通过使用直接抗球蛋白试验（DAT）结合溶血的临床和实验室检查结果来诊断的。修订后的指南提出了几个步骤来评估和诊断不明原因溶血且DAT阴性的患者，并且他们建议在评估冷凝集素滴度之前使用直接凝集试验（DAggT）筛查冷凝集素病（CAD）。利妥昔单抗正成为类固醇难治性温抗体型AIHA的首选二线治疗药物以及原发性CAD的一线治疗药物。在日本，利妥昔单抗是用于AIHA治疗的一种未获批准的药物，但未来将用作标准疗法。抗C1s抗体是一种已进入3期试验的用于CAD的新药。
