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
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。
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abstractst(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).
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