t(11;14)在系统性免疫球蛋白轻链淀粉样变性患者中的研究现状  

Research status on t(11;14)in patients with systemic immunoglobulin light chain amyloidosis

作　　者：韩雪[1] 陈烨[1] 陈文明[2] Han Xue;Chen Ye;Chen Wenming(Department of Hematology,Beijing Anzhen Hospital,Capital Medical University,Beijing 100029,China;Department of Hematology,Beijing Chao-Yang Hospital,Capital Medical University,Beijing 100020,China)

机构地区：[1]首都医科大学附属北京安贞医院血液科,北京100029 [2]首都医科大学附属北京朝阳医院血液科,北京100020

出　　处：《国际输血及血液学杂志》2024年第2期141-146,共6页International Journal of Blood Transfusion and Hematology

摘　　要：t(11;14)是系统性免疫球蛋白轻链淀粉样变性(AL)患者最常见的细胞遗传学异常,可引起细胞周期蛋白(CCN)D1和B细胞淋巴瘤(BCL)-2表达水平增加,从而导致克隆性浆细胞过量增殖。与伴其他细胞遗传学异常的AL患者相比,伴t(11;14)者具有特异性临床特征和治疗反应。目前,AL患者常用的临床治疗药物包括烷化剂、蛋白酶体抑制剂(PI)和免疫调节剂(IMID)等。CD38单克隆抗体和BCL-2抑制剂等新药对伴t(11;14)AL患者疗效良好。t(11;14)对AL患者治疗反应和预后的影响随着新药的应用不断更新。笔者拟对t(11;14)在AL发生中的作用机制,以及对患者临床特征和预后影响的研究现状进行阐述,旨在加深临床医师对伴t(11;14)AL的认识。t(11;14)is the most prevalent cytogenetic abnormality in patients with systemic immunoglobulin light chain amyloidosis(AL),which can cause increased expression of cyclin(CCN)D1 and B-cell lymphoma(BCL)-2,resulting in the overproliferation of clonal plasma cells.Compared with AL patients accompanied by other cytogenetic abnormalities,patients with t(11;14)have their own specific clinical characteristics and therapeutic responses.Current therapeutic agents for patients with AL include alkylating agents,proteasome inhibitors(PI),and immunomodulatory agents(IMID).New drugs,such as CD38 monoclonal antibody and BCL-2 inhibitors have shown good efficacy in treatment of AL patients with t(11;14).The effect of t(11;14)on prognosis and response to treatment in patients with AL is being updated.This article intends to elaborate on the latest research status on the role of t(11;14)in AL occurrence,and its impact on clinical characteristics and prognosis of patients with AL,with the aim of deepening clinicians′understanding of AL patients with t(11;14).

关 键 词：淀粉样变性 免疫球蛋白轻链淀粉样变性 浆细胞瘤 易位 遗传 预后 存活 t(11 14) 治疗 

分 类 号：R597.2[医药卫生—内科学]