Wing-Kwai and Alice Lee-Tsing Chung Transfusion Service, Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, US.
Division of Pediatric Hematology/Oncology, Department of Pediatrics, David Geffen School of Medicine at UCLA, Los Angeles, California, US.
Lab Med. 2022 Jul 4;53(4):e83-e86. doi: 10.1093/labmed/lmab101.
Most often, IgM-mediated autoimmune hemolytic anemia (AIHA) presents as cold agglutinin disease in the pediatric population. The IgM warm agglutinins are rare, with few reports in the literature. This case study describes a 5 year old girl with nausea, abdominal pain and jaundice, and a hemoglobin of 5.5 g/dL who was diagnosed with a warm reactive IgM AIHA. The laboratory workup revealed a pan-reactive antibody and a direct antiglobulin test negative for IgG and C3. A thermal amplitude assay revealed reactive IgM antibodies at 37°C, 30°C, 25°C, and 4°C and an antibody titer of 1:8. An adsorption for IgM-specific autoantibodies exposed underlying anti-E and anti-Cw alloantibodies. Transfusion of phenotypically matched red blood cell units supported ongoing hemolysis. The AIHA treatment included steroids followed by rituximab with complete resolution. A literature review shows variable outcomes for warm AIHA in the pediatric population and often describes the presence of warm reactive IgM-mediated AIHA as an indicator for poor prognosis.
大多数情况下，IgM 介导的自身免疫性溶血性贫血（AIHA）在儿科人群中表现为冷凝集素病。IgM 温反应性抗体较为罕见，文献报道较少。本病例研究描述了一名 5 岁女孩，她出现恶心、腹痛和黄疸，血红蛋白为 5.5g/dL，被诊断为温反应性 IgM AIHA。实验室检查显示全反应性抗体和直接抗球蛋白试验阴性 IgG 和 C3。热振幅测定显示在 37°C、30°C、25°C 和 4°C 时反应性 IgM 抗体，抗体滴度为 1:8。IgM 特异性自身抗体的吸附揭示了潜在的抗-E 和抗-Cw 同种异体抗体。输注表型匹配的红细胞单位支持持续溶血。AIHA 的治疗包括类固醇，随后是利妥昔单抗，完全缓解。文献回顾显示儿科人群中温型 AIHA 的结果各不相同，常将温反应性 IgM 介导的 AIHA 作为预后不良的指标。
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