Department of Medical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing Key Laboratory of Clinical Study on Anticancer Molecular Targeted Drugs, Beijing, 100021, China.
Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100021, China.
J Cancer Res Clin Oncol. 2023 Sep;149(11):8483-8494. doi: 10.1007/s00432-023-04714-1. Epub 2023 Apr 24.
PURPOSE: This study aims to investigate the clinical and molecular differences between diffuse large B-cell lymphoma (DLBCL) patients with MYD88 and MYD88. METHODS: DLBCL patients with MYD88 variations were collected from the Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College (CHCAMS), and Suzhou Municipal Hospital from February 6th, 2007 to May 20th, 2022. Clinicopathological parameters and treatment outcomes between MYD88 and MYD88 were investigated. RESULTS: A total of 132 patients with MYD88 variations from a cohort of 475 DLBCL patients were included, among which, 78 were MYD88, while 54 were MYD88. MYD88 was more common in non-GCB subtype than MYD88 (83% vs. 60%, P = 0.004). Besides, MYD88 was significantly related to higher proportion of testicle/ central nervous system involvement (31% vs. 6%, P < 0.001), PIM1 mutation (71% vs. 39%, P < 0.001), and PIM1 hypermutation (28% vs. 11%, P = 0.018), compared with MYD88. Compared with MYD88, MYD88 were more likely to have higher percentage of advanced stage (60% vs. 42%, P = 0.044), extranodal site ≥ 2 (45% vs. 28%, P = 0.044), elevated LDH (55% vs. 35%, P = 0.033), positive CD10 expression (36% vs. 16%, P = 0.009), BCL-6 translocation (20% vs. 8%, P = 0.033), and NOTCH pathway gene alteration (24% vs. 13%, P = 0.040). In non-GCB DLBCL subtype, patients with MYD88 were significantly associated with worse progression free survival (PFS) than those with MYD88 when treated initially with R-CHOP/R-CHOP-like regimen (P = 0.010). CONCLUSION: The findings of this study indicate that DLBCL patients with MYD88 and MYD88 are likely to be two subgroups with different clinical and molecular characteristics. The survival of patients with MYD88 is not superior than those with MYD88, even poorer when focusing on the non-GCB subtype.
目的：本研究旨在探讨具有 MYD88 变异的弥漫性大 B 细胞淋巴瘤（DLBCL）患者的临床和分子差异。
方法：收集了 2007 年 2 月 6 日至 2022 年 5 月 20 日期间，中国医学科学院北京协和医学院肿瘤医院和苏州市立医院的具有 MYD88 变异的 DLBCL 患者。研究了 MYD88 和 MYD88 患者之间的临床病理参数和治疗结局。
结果：在 475 例 DLBCL 患者的队列中，共纳入了 132 例具有 MYD88 变异的患者，其中 78 例为 MYD88，54 例为 MYD88。与 MYD88 相比，MYD88 更常见于非生发中心 B 细胞（GCB）亚型（83%比 60%，P=0.004）。此外，与 MYD88 相比，MYD88 与更高比例的睾丸/中枢神经系统受累（31%比 6%，P<0.001）、PIM1 突变（71%比 39%，P<0.001）和 PIM1 超突变（28%比 11%，P=0.018）显著相关。与 MYD88 相比，MYD88 更有可能处于晚期（60%比 42%，P=0.044）、结外部位≥2 个（45%比 28%，P=0.044）、LDH 升高（55%比 35%，P=0.033）、CD10 表达阳性（36%比 16%，P=0.009）、BCL-6 易位（20%比 8%，P=0.033）和 NOTCH 通路基因改变（24%比 13%，P=0.040）的比例更高。在非 GCB DLBCL 亚型中，与 MYD88 相比，初始接受 R-CHOP/R-CHOP 样方案治疗的患者，MYD88 患者的无进展生存期（PFS）明显较差（P=0.010）。
结论：本研究结果表明，具有 MYD88 和 MYD88 的 DLBCL 患者可能是具有不同临床和分子特征的两个亚群。与 MYD88 相比，MYD88 患者的生存情况并不优于 MYD88 患者，尤其是在非 GCB 亚型中。
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