慢性淋巴细胞白血病中B细胞受体同型模式的生物学特征和临床意义  

Biological characteristics and clinical significance of stereotyped B-cell receptor in chronic lymphocytic leukemia

作　　者：邱彤璐 李建勇 夏奕 Qiu Tongu;Li Jianyong;Xia Yi(Department of Hematology,Key Laboratory of Nanjing Medical University,the First Afiliated Hospital of Nanjing Medical University,Jiangsu Province Hospital,Collaborative Innovation Center for Cancer Personalized Medicine,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,南京医科大学血液研究重点实验室,江苏省肿瘤个体化医学协同创新中心,南京210029

出　　处：《中华血液学杂志》2024年第2期197-202,共6页Chinese Journal of Hematology

基　　金：国家自然科学基金(81700193、82170186);中国博士后自然科学基金(2021M691336);江苏省博士后自然科学基金(2021K083A);江苏省科教能力提升工程(ZDXK202209)。

摘　　要：慢性淋巴细胞白血病(CLL)是西方成人最常见的白血病,尽管CLL在亚洲人种中发病率偏低,但随着人口老龄化,CLL发病率在我国呈上升趋势。CLL细胞与微环境的交互中,B细胞受体免疫球蛋白(B-cell receptor immunoglobulin,BCR IG)对抗原的识别起重要作用,免疫球蛋白重链可变区(IG heavy variable region,IGHV)的突变状态更是判断CLL预后的经典标志物。超过40%的CLL患者间存在IGHV的偏向性使用和高度相似的重链可变区互补决定区3(heavy complementarity-determining region 3,HCDR3)氨基酸片段,此现象称为同型模式BCR。不同亚群的同型模式BCR存在独特的生物学和临床特征。其中IGHV有突变但预后差的#2亚群和发生Richter转化风险高的#8亚群被欧洲CLL研究启动组推荐纳入IGHV突变状态的临床报告之中。本文综述CLL中同型模式BCR的定义、分布、生物学特征和临床意义。Chronic lymphocytic leukemia(CLL)is the most common leukemia in the Western.adults,although the incidence of CLL is relatively low in Asian populations.However,with the aging population,the incidence of CLL is increasing in China.The interaction between CLL cells and the microenvironment plays a crucial role in the recognition of antigens by the B-cell receptor immunoglobulin(BCR IG).The mutational status of the immunoglobulin heavy variable region(IGHV)is a classical prognostic marker for CLL.Over 40%of CLL patients exhibit biased usage of IGHV and highly similar amino acid sequences in the heavy complementarity-determining region 3(HCDR3),known as the BCR stereotypy.Different subgroups of stereotyped BCR exhibit distinct biological and clinical features.Among them,subset#2 with mutated IGHV and poor prognosis,as well as the subset#8 with a high risk of Richter transformation,have been recommended by the European Research Initiative on CLL to be included in clinical reports on IGHV mutational status.This review summarizes the definition,distribution,biological characteristics,and clinical significance of clonality patterns of the BCR in CLL.

关 键 词：慢性淋巴细胞白血病 B细胞受体 互补决定区3 RICHTER 免疫球蛋白 偏向性 重链可变区 临床报告 

分 类 号：R733.72[医药卫生—肿瘤]