MGCS: where do we stand today?

doi:10.1182/hematology.2024000572

2024-12-06

PRE

David Iberri

Michaela Liedtke

DOI：10.1182/hematology.2024000572

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临床意义单克隆丙种球蛋白病（MGCS）是一组异质性障碍，其特征为存在一个惰性B细胞或浆细胞克隆，该克隆产生毒性单克隆免疫球蛋白，导致终末器官功能障碍。MGCS是一种临床病理学诊断，需要在正确的临床背景下证明存在单克隆免疫球蛋白。最常见的MGCS综合征为肾脏、神经和皮肤表现，尽管血液系统和多器官MGCS综合征也日益受到关注。治疗通常针对基础克隆群体；针对免疫球蛋白的治疗以及补体和细胞因子拮抗剂已应用于特定MGCS综合征，并可能在部分患者中起到暂时缓解作用。其他章节回顾肾脏和神经MGCS；本章重点介绍血液系统和多器官MGCS综合征。

Keyword:

MONOCLONAL GAMMOPATHY

SCHNITZLERS SYNDROME

PREVALENCE

期刊

Hematology and American Society of Hematology Education Program

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