Objective: To investigate the clinical features and prognosis of MYC/BCL2 double-expression diffuse large B-cell lymphoma (DEL) . Methods: The clinical data, including clinical characteristics, survival, and prognostic factors, of 166 patients with DEL treated at Peking University Third Hospital from January 2016 to December 2020 were retrospectively analyzed. Results: A total of 410 patients with diffuse large B-cell lymphoma were collected, including 166 cases (40.5%) of DEL. There were 82 males and 84 females with a median age of 63.5 (21-95) years at diagnosis. A total of 110 patients (66.3%) were aged over 60 years at initial diagnosis, 106 patients (106/163, 65.0%) had elevated lactate dehydrogenase (LDH) at diagnosis, 74 patients (74/160, 46.2%) had β(2) microglobulin level over 3 mg/L at diagnosis, and 107 patients (107/163, 65.6%) had≥2 extranodal involvement. Sixty-five patients (65/166, 39.2%) had B symptoms, 131 patients (131/165, 79.4%) had stage Ⅲ and Ⅳ disease at initial diagnosis, 41 patients (41/161, 25.5%) had an International Prognostic Index (IPI) score of 0-2 at initial diagnosis, and 38 patients (38/161, 23.6%) had an IPI score of 3 at initial diagnosis. Eighty-two patients (82/161, 50.9%) had an IPI score of 4-5 at initial diagnosis. Nine (9/56, 16.1%) patients with DEL had MYD88 and CD79B mutations. Univariate analysis showed that age over 60 years (P=0.004) , increased β(2) microglobulin level (P=0.002) , and high IPI score (P=0.003) were associated with poor overall survival (OS) . Increased β(2) microglobulin level (P=0.031) , LDH level (P=0.017) , stage Ⅲ-Ⅳ (P=0.001) , high IPI score (P=0.013) , immunohistochemical p53 mutation (P=0.049) , and PIM1 mutation (P=0.039) were associated with poor progression-free survival (PFS) . Multivariate analysis showed that IPI score of 4-5 was an independent risk factor for the prognosis of DEL (HR=2.622, 95% CI 1.398-4.917, P=0.003) . Survival analysis showed that there was a significant difference in the PFS between patients with DEL and those without DEL (65.6% vs 75.1%, P=0.002) . However, there was no significant difference in the OS (81.8% vs 83.6%, P=0.226) . In patients with DEL, the overall response rate of R-EPOCH regimen was higher than that of RCHOP or RCHOP-like regimen (81.5% vs 63.4%, P=0.004) . Conclusion: DEL is a group of aggressive lymphomas with relatively poor PFS. The R-EPOCH regimen may improve the overall prognosis of patients.
目的： 探讨双表达弥漫大B细胞淋巴瘤（DLBCL）的临床特征与预后。 方法： 回顾性分析2016年1月至2020年12月北京大学第三医院收治的166例双表达DLBCL患者的临床资料，分析患者的临床特征、生存和预后因素。 结果： 共收集DLBCL患者410例，其中双表达淋巴瘤（DEL）166例（40.5%）。男82例，女84例，中位诊断年龄63.5（21~95）岁。110例（66.3%）患者初诊年龄≥60岁，106例（106/163，65.0%）患者诊断时LDH升高，74例（74/160，46.2%）诊断时β(2)-微球蛋白（β(2)-MG）≥ 3 mg/L，107例（107/163，65.6%）结外受累数目≥2个，65例（65/166，39.2%）有B症状，131例（131/165，79.4%）初诊时分期为Ⅲ、Ⅳ期，41例（41/161，25.5%）初诊时国际预后指数（IPI）评分0~2分，38例（38/161，23.6%）初诊时IPI评分3分，82例（82/161，50.9%）初诊IPI评分4~5分。DEL患者中9例（9/56，16.1%）具有MYD88和CD79B突变。单因素分析显示，年龄≥60岁（P=0.004）、β(2)-MG水平升高（P=0.002）、IPI评分高（P=0.003）与较差的总生存（OS）相关，β(2)-MG水平升高（P=0.031）、LDH水平升高（P=0.017）、分期Ⅲ~Ⅳ期（P=0.001）、IPI评分高（P=0.013）、免疫组化p53突变型（P=0.049）和PIM1突变（P=0.039）与较差的无进展生存（PFS）相关。多因素分析显示，IPI评分4~5分（HR=2.622，95%CI 1.398~4.917，P=0.003）是影响DEL患者OS的独立危险因素。生存分析显示，DEL与非DEL患者的PFS率差异有统计学意义（65.6%对75.1%，P=0.002），而OS率的差异无统计学意义（81.8%对83.6%，P=0.226）。在DEL患者中，REPOCH方案的总有效率高于RCHOP或RCHOP样方案（81.5%对63.4%，P=0.004）。 结论： DEL是一组侵袭性较强的淋巴瘤，具有较差的PFS，采用REPOCH方案化疗可能会改善患者的整体预后。.
Keywords: Clinical features; Double expression lymphoma; Gene, BCL2; Lymphoma, large B-cell, diffuse; Prognosis.