摘要
原发性睾丸淋巴瘤(PTL)是一种少见的侵袭性结外淋巴瘤,以无痛性单侧睾丸肿块为典型临床特点。PTL患者预后差,极易出现中枢神经系统(CNS)和对侧睾丸复发。利妥昔单抗对于改善PTL患者生存和预后的作用仍存在争议,因此亟需新的治疗方案。近年来,借助高速发展的二代测序(NGS)技术,在PTL中发现髓样分化因子(MYD)88、CD79B、莫洛尼小鼠白血病病毒前病毒整合位点(PIM)1和转导素β1X连锁受体蛋白(TBL1XR)1等高频突变基因,这些基因突变可能通过激活核因子-κB信号通路介导PTL的发生、发展。因此,笔者拟通过对PTL中的核因子-κB信号通路及高频基因突变进行综述,旨在探究PTL的发病机制,为寻找有效且精准的PTL治疗靶点提供参考,以期改善PTL患者预后。
Primary testicular lymphoma(PTL)is a rare and aggressive extranodal lymphoma characterized by a painless unilateral testicular mass.Patients with PTL have a poor prognosis and are highly susceptible to central nervous system(CNS)and contralateral testicular recurrence.However,role of rituximab in improving survival and prognosis of PTL patients are still controversial,so new treatment options are urgently needed.In recent years,the next generation sequencing(NGS)technology has developed rapidly.With help of NGS technology,high-frequency mutant genes such as myeloid differentiation factor(MYD)88,CD79B,provirus integration site for moloney murine leukemia virus(PIM)1 and transducin beta-like 1 X-linked receptor(TBL1XR)1 are found in PTL,which may mediate occurrence and development of PTL by activating nuclear factor-κB signaling pathway.Therefore,by introducing nuclear factor-κB signaling pathway and high-frequency gene mutations in PTL,this article aims to review pathogenesis of PTL,in order to discover effective and precise therapeutic targets to improve prognosis of PTL patients.
作者
王昀哲
何翠颖
王连静
李炜静
刘玮
刘丽宏
Wang Yunzhe;He Cuiying;Wang Lianjing;Li Weijing;Liu Wei;Liu Lihong(Department of Hematology,Fourth Hospital of Hebei Medical University,Shijiazhuang 050011,Hebei Province,China)
出处
《国际输血及血液学杂志》
CAS
2021年第6期547-552,F0003,共7页
International Journal of Blood Transfusion and Hematology
基金
希思科-石药肿瘤研究基金项目(Y-sy2018-046、Y-sy2018-047)。
