初治弥漫大B细胞淋巴瘤患者MYD88基因突变的临床特征及预后分析

目的:探讨MYD88基因突变对初治弥漫大B细胞淋巴瘤（diffuse large B-cell lymphoma，DLBCL）的临床特征及预后的影响。方法:收集2013年1月至2015年1月空军军医大学第二附属医院血液科的74例初治DLBCL患者，回顾分析MYD88突变组（MYD88mut）和MYD88野生组（MYD88wt）患者的临床特征及治疗效果。结果:74例DLBCL患者中，MYD88mut组和MYD88wt组患者性别、年龄、ECOG评分、LDH、Ann Arbor分期、结外侵犯及IPI评分比较差异无统计学意义。MYD88mut组主要为ABC亚型（75.00%）。12例MYD88mut突变均为错义突变，其中8例氨基酸改变为L265P。MYD88mut组2周期R-CHOP方案完全缓解率（complete remission,CR）为41.67%，部分缓解率(partial remission,PR)为33.33%,客观有效率(objective effective,OR)为75.00%。MYD88wt组分别为77.42%、11.29%和88.71%。2组比较，2周期CR率具有统计学差异（P=0.030 4）。生存分析结果显示，MYD88mut组及MYD88wt组患者5年OS分别为54.98%与73.53%，PFS分别为48.61%与66.54%，2组比较，OS及PFS均具有统计学差异（P=0.003 4，P=0.031 9）。结论:MYD88突变主要存在于DLBCL的ABC亚型，且MYD88突变提示其预后不良。

Analysis of clinical characteristics and prognosis of MYD88 gene mutation in patients with primary diffuse large B-cell lymphoma

Objective:To discuss the effect of MYD88 gene mutation on the clinical characteristics and prognosis in patients with diffuse large B-cell lymphoma（DLBCL).Methods:74 patients with DLBCL were selected from the Department of Hematology,Second Affiliated Hospital of Air Force Military Medical University from January 2013 to January 2015.The clinical basic characteristics and therapeutic effects of the MYD88 mutation group and MYD88 normal group were reviewed.Results:Among the 74 patients with DLBCL,there was no significant difference in gender,age,ECOG score,LDH,Ann Arbor stage,extranodal invasion and IPI score between the MYD88wt group and MYD88mut.The main subtypes were ABC subtypes in MYD88mut group（75.00%).The results of DLBCL MYD88 sequencing of 12 cases of MYD88mut showed that all the 12 mutations were missense mutations.In MYD88mut group,the complete remission rate,partial remission rate and total remission rate were 41.67%,33.33% and 75.00% after 2 cycles of R-CHOP therapy,respectively.In MYD88wt group,the CR of R-CHOP regimen in 2 cycles was 77.42%,the PR rate was 11.29%,and the OR rate was 88.71%.Compared with the two groups,there was significant difference in CR rate between the two groups（P=0.030 4).The results of survival analysis showed that the 5-year OS of MYD88mut group and MYD88wt group were 54.98% and 73.53%,and 5-year PFS were 48.61% and 66.54%,respectively.There was significant difference between the two groups（P=0.003 4,P=0.031 9).Conclusion:MYD88 mutation mainly exists in ABC subtypes of DLBCL,and MYD88 mutation indicates poor prognosis.