摘要目的:探讨重度温抗体型自身免疫性溶血性贫血(warm autoimmune hemolytic anemia，w-AIHA)的临床特征、疗效及临床干预策略选择。方法:回顾性分析2007年6月至2019年3月在首都儿科研究所附属儿童医院血液科住院治疗的21例w-AIHA患儿的临床特点、治疗方案和疗效。结果:21例重度w-AIHA患儿平均年龄8.0(2.5，20.0)个月，随访时间33.0(18.5，110.0)个月。10例(47.6%)患儿血红蛋白低于30 g/L，5例(23.8%)患儿表现为Evans综合征，5例(23.8%)患儿为继发性病例，9例(42.8%)患儿有感染史，2例患儿摄入花粉诱发；溶血危象5例(26.3%)，12例(57.1%)存在配血困难，8例(38.1%)入住ICU，5例(23.8%)出现休克。所有患儿均使用了皮质类固醇和静脉用丙种球蛋白，16例(76.2%)患儿予以二线方案(环磷酰胺、利妥昔单抗等)治疗，15例完全缓解，3例部分缓解，3例无效及死亡。结论:感染仍是儿童重度w-AIHA病例的重要诱发因素，继发性病例比例高，继发病因以免疫缺陷病为主，溶血危象发生率高，且存在配血、输血困难，成功输血为抢救成功的关键因素。重度w-AIHA患儿应早期入住ICU密切监测，利妥昔单抗需提前应用，保证成功输血。

abstractsObjective:To explore the clinical manifestation, efficacy and treatment strategies in patients with severe warm autoimmune hemolytic anemia (w-AIHA).Methods:A total of 21 patients with w-AIHA who were hospitalized in Children′s Hospital of Capital Institute of Pediatrics from June 2007 to March 2019 were included, and the clinical characteristics, treatment strategies and responses were retrospectively analyzed.Results:A total of 21 children with severe w-AIHA had an average age of 8.0 (2.5, 20.0) months and a follow-up time of 33.0 (18.5, 110.0) months.In 10 (47.6%) cases, the hemoglobin levels were lower than 30 g/L.Evans′ syndrome was diagnosed in five(23.8%) cases.Five (23.8%) cases were secondary cases.Nine (42.8%) cases had a previous infection history and two cases were pollen-induced.Five (23.8%) cases had hemolytic crisis.A total of 12 (57.1%) cases had cross-matching difficulty.Eight (38.1%) cases were admitted to the ICU, and five (23.8%) cases had shock.All children received corticosteroids and intravenous immunoglobulin, 16 (76.2%) cases were treated with second-line regimens (cyclophosphamide and rituximab, etc.), 15 cases had complete response, three cases had partial response and three cases had no response and died.Conclusion:Infection is an important predisposing factor in children with severe w-AIHA, and secondary cases have a higher proportion, mainly caused by immunodeficiency disease.Patients tend to have a high incidence of hemolytic crisis and have difficulty in matching and transfusion.Therefore, transfusion is the key for successful rescue.It is suggested that children with severe w-AIHA require ICU admission for early monitoring and rituximab should be applied in advance to ensure successful transfusion.