原发性中枢神经系统淋巴瘤的临床特征及预后分析  

Clinical characteristics and prognostic analysis of primary central nervous system lymphoma

作　　者：米倩 白斯君[1,2] 耿晔 高轶男[1,2] 张彩霞 贺建霞 MI Qian;BAI Sijun;GENG Ye;GAO Yinan;ZHANG Caixia;HE Jianxia(The Fifth Clinical Medical College of Shanxi Medical University,Shanxi Taiyuan 030012,China;Hematology Department,Shanxi Provincial People's Hospital,Shanxi Taiyuan 030012,China)

机构地区：[1]山西医科大学第五临床医学院,山西太原030012 [2]山西省人民医院血液科,山西太原030012

出　　处：《现代肿瘤医学》2023年第6期1112-1117,共6页Journal of Modern Oncology

基　　金：山西省自然科学基金(编号:201901D111435)。

摘　　要：目的:探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma, PCNSL)患者的临床病理和分子学特征及预后影响因素。方法:收集2013年03月至2021年05月山西省人民医院35例PCNSL患者的临床资料及组织标本,总结其临床病理特征并应用二代测序技术检测淋巴瘤相关基因,分析年龄、病灶部位、乳酸脱氢酶(lactate dehydrogenase, LDH)、免疫组化等指标对预后的影响,比较不同基因突变组及治疗方案之间的总生存(overall survival, OS)差异。结果:最常见的基因突变为MYD88(66.7%,12/18),IGLL5、PIM1(38.9%,7/18);PIM1突变组、IGLL5突变组OS差于未突变组,差异均有统计学意义(P=0.019和0.021);多因素Cox回归分析显示LDH、不同的治疗方案是PCNSL患者独立的预后影响因素(P=0.007和0.002);接受大剂量甲氨蝶呤(methotrexate, MTX)、利妥昔单抗(rituximab, R)治疗组OS明显优于未接受组,差异均有统计学意义(P=0.005和<0.001);使用布鲁顿酪氨酸激酶抑制剂(broughton tyrosine kinase inhibitor, BTKi)组与未接受组相比,有生存获益的趋势,但未显示出统计学差异。结论:血清LDH升高与不良预后显著相关,PIM1及IGLL5突变患者生存较差,含MTX、R治疗方案可改善患者生存。Objective:To explore the clinicopathological and molecular features and prognostic factors in patients with primary central nervous system lymphoma(PCNSL).Methods:Collect clinical data and tissues amples of 35 patients with PCNSL in Shanxi Provincial People’s Hospital from March 2013 to May 2021,summarize the clinicopathological characteristics and apply second-generation sequencing technology to detect lymphoma-related genes.The effects of age, lesion location, lactate dehydrogenase(LDH) and immunohistochemistry on prognosis were analyzed, and the differences in overall survival(OS) in different gene mutation groups and treatment regimens were compared.Results:The most universal gene mutation was MYD88(66.7%,12/18),IGLL5,PIM1(38.9%,7/18).The OS of PIM1 mutation group and IGLL5 mutation group was significantly lower than that of the non-mutation group(P=0.019 and 0.021).Multivariate Cox regression analysis showed that LDH and different treatment regimens were independent prognostic factors of PCNSL patients(P=0.007 and 0.002).The OS of the group receiving high-dose methotrexate(MTX) and rituximab(R) was significantly better than the untreated group(P=0.005 and <0.001).There was a trend of survival benefit in the group using broughton tyrosine kinase inhibitor(BTKi) compared with the group not receiving it, but there was no statistical difference.Conclusion:Elevated serum LDH was significantly associated with poor prognosis.The patients with PIM1 and IGLL5 mutations had inferior survival.The treatment with MTX and R can improve the survival of patients.

关 键 词：中枢神经系统 弥漫大B细胞淋巴瘤 MYD88 PIM1 预后 

分 类 号：R733.4[医药卫生—肿瘤]