[冷凝集素病:病理学、诊断和治疗]。

Hideho Wada
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摘要

冷凝集素病(CAD)是一种由经典补体依赖途径介导的免疫性溶血性疾病,约占自身免疫性溶血性贫血(AIHA)病例的 8%。原发性 CAD 是一种骨髓克隆性 B 细胞淋巴细胞增生性疾病,会产生 IgM 型-M 蛋白,而传统的继发性 CAD 是冷凝集素综合征(CAS)。临床表现大致可分为溶血导致的慢性贫血,以及寒冷环境下红细胞聚集导致的外周循环衰竭相关症状。并非所有患者都需要药物治疗,但前者首选针对补体 C1s 的单克隆抗体治疗,后者首选 B 细胞抑制剂。由于冷性 AIHA 与温性 AIHA 的治疗方法不同,因此误诊会严重影响治疗效果。血液检测最重要的一点是标本的温度控制。如果在血清分离前没有将血清温度控制在 37-38°C 之间,冷凝集素滴度、IgM 定量、电泳和免疫固定方法可能会产生假阴性结果。正确处理标本,同时了解 CAD 的各种临床特征,将有助于正确诊断和适当治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Cold agglutinin disease: pathology, diagnosis, and treatment].

Cold agglutinin disease (CAD), an immune hemolytic disease mediated by the classical complement-dependent pathway, accounts for approximately 8% of autoimmune hemolytic anemia (AIHA) cases. Primary CAD is a clonal B-cell lymphoproliferative disease of the bone marrow that produces IgM type-M protein, while conventional secondary CAD is cold agglutinin syndrome (CAS). Clinical findings are broadly classified into chronic anemia due to hemolysis and symptoms associated with peripheral circulatory failure due to erythrocyte aggregation under cold exposure. Not all patients require drug therapy, but monoclonal antibody therapy against complement C1s is preferred for the former presentation and B-cell suppressors for the latter. As cold AIHA is treated differently than warm AIHA, misdiagnosis can significantly impact the outcome of treatment. The most important aspect of blood testing is temperature control of specimens. Cold agglutinin titer, IgM quantification, electrophoresis, and immunofixation methods may produce false-negative results if the serum is not temperature-controlled at 37-38°C until serum separation. Correct handling of specimens, along with knowledge of the various clinical features of CAD, will lead to correct diagnosis and appropriate treatment.

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