华氏巨球蛋白血症研究进展  

Progress of Waldenstrom macroglobulinemia

作　　者：何杰 王莉[1] 徐卫[1] He Jie;Wang Li;Xu Wei(Department of Hematology,the First Affiliated Hospital with Nanjing Medical University,Jiangsu Province Hospital,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院江苏省人民医院血液科,210029

出　　处：《白血病．淋巴瘤》2020年第1期20-22,共3页Journal of Leukemia & Lymphoma

摘　　要：华氏巨球蛋白血症(WM)是一种少见的、难以治愈的以血清单克隆IgM为主要特征的惰性淋巴瘤,全基因组测序显示MYD88和CXCR4基因突变是WM最常见的分子遗传学改变。近年来随着二代测序等技术的发展,对WM发生机制的研究不断深入,同时,Bruton酪氨酸激酶(BTK)抑制剂、蛋白酶体抑制剂等新型药物的临床试验不断开展,使WM的预后得到了改善。文章主要就第61届美国血液学会年会关于WM治疗、预后分析等的最新研究进展进行介绍。Waldenstrom macroglobulinemia(WM)is a rare and incurable indolent lymphoma,characterized by serum monoclonal IgM.Whole-genome sequencing has showed that MYD88 and CXCR4 gene mutations are the most common molecular genetic changes in WM.In recent years,with the development of next-generation sequencing and other technologies,the research on the pathogenetic mechanism of WM has been continuously explored.Clinical trials of Bruton tyrosine kinase(BTK)inhibitors,proteasome inhibitors and other new drugs have been continuously carried out,improving the prognosis of WM.This paper reviews the latest research on treatment and prognosis analysis of WM in the 61st American Society of Hematology Annual Meeting.

关 键 词：WALDENSTROM巨球蛋白血症 突变 谷胱甘肽 蛋白激酶抑制剂 蛋白酶抑制药 预后 

分 类 号：R73[医药卫生—肿瘤]