临床荟萃

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华氏巨球蛋白血症诊断和治疗

  

  1. 安徽医科大学第一附属医院 血液科,安徽 合肥 230001
  • 出版日期:2019-06-20 发布日期:2019-08-02
  • 通讯作者: 刘沁华, Email: 1740699661@qq.com
  • 作者简介:刘沁华,医学博士,副主任医师。中国抗癌协会血液肿瘤专业委员会青年委员,中国老年学会血液学分会淋巴瘤学组委员,安徽省医学会血液学分会委员兼秘书,安徽省医学会血液学分会血栓与止血学组副组长,安徽省全科医师协会血液病学分会理事。从事血液病专业多年,对血液病常见病、多发病及疑难杂症、危急重症的诊治有着较丰富的临床经验。目前专业方向为淋巴瘤、骨髓瘤的诊断治疗。

Diagnosis and treatment  of Waldenstrm's macroglobulinaemia

  1. Department of Hematology,  the First Affiliated Hospital of Anhui Medical University,  Hefei 230001, China
  • Online:2019-06-20 Published:2019-08-02
  • Contact: Corresponding author: Liu Qinhua, Email: 1740699661@qq.com

摘要: 华氏巨球蛋白血症(Waldenstrm’s macroglobulinemia,  WM)/淋巴浆细胞淋巴瘤 (lymphoplasmacytic lymphoma,  LPL)是一种少见的惰性B细胞非霍奇金淋巴瘤,该病临床以淋巴浆细胞骨髓、脏器浸润和血清单克隆免疫球蛋白M水平升高为特点。近年来,随着免疫学、分子遗传学的发展,以及新药的不断涌现,WM/LPL 的诊断与治疗取得了较大的进展,本文将对WM/LPL 的诊断与治疗进行相关综述。

关键词: Waldenstrom巨球蛋白血症, 淋巴瘤,  , 小细胞, 免疫球蛋白M

Abstract: Waldenstrm's macroglobulinemia (WM)/lymphoplasmictic lymphoma (LPL) is a rare,  incurable Bcell lymphoma. Its clinical course  is characterized by elevated immunoglobulinM (IgM) levels,  lymphoplasmacytic bone marrow  infiltration with cytopenia,  hepatomegaly,  splenomegaly,  and lymphadenopathy.Recently,  great progress has been made to better explain the pathobiology of WM,  which has  identified several clinical and genetic markers that serve prognosticate disease course and  patient outcomes. This article reviews some of the recent advance with  respect to prognostic markers and new treatments that are currently in progress.

Key words: Waldenstrm macroglobulinemia, lymphoplasm, , smallcell, immunoglobulin M