Tang S H, Tian L, Zhao W, Wang J, Ke X Y
Department of Hematology, Peking University Third Hospital, Beijing 100083, China.
Zhonghua Xue Ye Xue Za Zhi. 2022 Sep 14;43(9):771-777. doi: 10.3760/cma.j.issn.0253-2727.2022.09.010.
To investigate the clinical features and prognosis of MYC/BCL2 double-expression diffuse large B-cell lymphoma (DEL) . 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. 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 (=0.004) , increased β(2) microglobulin level (=0.002) , and high IPI score (=0.003) were associated with poor overall survival (OS) . Increased β(2) microglobulin level (=0.031) , LDH level (=0.017) , stage Ⅲ-Ⅳ (=0.001) , high IPI score (=0.013) , immunohistochemical p53 mutation (=0.049) , and PIM1 mutation (=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 (=2.622, 95% 1.398-4.917, =0.003) . Survival analysis showed that there was a significant difference in the PFS between patients with DEL and those without DEL (65.6% 75.1%, =0.002) . However, there was no significant difference in the OS (81.8% 83.6%, =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% 63.4%, =0.004) . DEL is a group of aggressive lymphomas with relatively poor PFS. The R-EPOCH regimen may improve the overall prognosis of patients.
探讨MYC/BCL2双表达弥漫性大B细胞淋巴瘤（DEL）的临床特征及预后。回顾性分析2016年1月至2020年12月在北京大学第三医院接受治疗的166例DEL患者的临床资料，包括临床特征、生存情况及预后因素。共收集410例弥漫性大B细胞淋巴瘤患者，其中DEL患者166例（40.5%）。诊断时男性82例，女性84例，中位年龄63.5（21 - 95）岁。初诊时110例患者（66.3%）年龄超过60岁，106例患者（106/163，65.0%）诊断时乳酸脱氢酶（LDH）升高，74例患者（74/160，46.2%）诊断时β2微球蛋白水平超过3mg/L，107例患者（107/163，65.6%）有≥2个结外受累。65例患者（65/166，39.2%）有B症状，131例患者（131/165，79.4%）初诊时为Ⅲ期和Ⅳ期疾病，41例患者（41/161，25.5%）初诊时国际预后指数（IPI）评分为0 - 2，38例患者（38/161，23.6%）初诊时IPI评分为3。82例患者（82/161，50.9%）初诊时IPI评分为4 - 5。9例（9/56，16.1%）DEL患者有MYD88和CD79B突变。单因素分析显示，年龄超过60岁（P = 0.004）、β2微球蛋白水平升高（P = 0.002）及IPI评分高（P = 0.003）与总生存期（OS）差相关。β2微球蛋白水平升高（P = 0.031）、LDH水平（P = 0.017）、Ⅲ - Ⅳ期（P = 0.001）、IPI评分高（P = 0.013）、免疫组化p53突变（P = 0.049）及PIM1突变（P = 0.039）与无进展生存期（PFS）差相关。多因素分析显示，IPI评分为4 - 5是DEL预后的独立危险因素（P = 2.622，95%CI 1.398 - 4.917，P = 0.003）。生存分析显示，DEL患者与非DEL患者的PFS有显著差异（65.6%对75.1%，P = 0.002）。然而，OS无显著差异（81.8%对83.6%，P = 0.226）。在DEL患者中，R - EPOCH方案的总缓解率高于RCHOP或RCHOP类方案（81.5%对63.4%，P = 0.004）。DEL是一组侵袭性淋巴瘤，PFS相对较差。R - EPOCH方案可能改善患者的总体预后。
