弥漫性大 B 细胞淋巴瘤 (DLBCL) 是最常见的非霍奇金淋巴瘤 (NHL)，具有显着的异质性。大约 5-10% 的 DLBCL 表达 CD5，这使得 CD5 DLBCL 成为罕见的亚组。不同的研究表明，CD5 DLBCL 患者通常年龄较大，为女性，乳酸脱氢酶水平较高，东部肿瘤合作组 (ECOG) 表现状态 > 1，以及较高的国际预后指数 (IPI) 评分。此外，患者通常处于晚期疾病，中枢神经系统（CNS）复发和骨髓受累的发生率很高。 CD5 DLBCL 细胞更可能表达 MYC、BCL-2 和 MUM-1，不太可能表达 CD10，并且大多数属于活化 B 细胞样 (ABC) 亚型。 CD5 DLBCL 患者预后不良的潜在机制可能与 CD5 介导的 B 细胞受体 (BCR) 依赖性和非依赖性途径有关。传统利妥昔单抗联合环磷酰胺、阿霉素、长春新碱和泼尼松（R-CHOP）方案对 CD5 DLBCL 患者的疗效并不令人满意。尽管有回顾性研究的支持证据，但目前尚不清楚剂量调整的依托泊苷、泼尼松、长春新碱、环磷酰胺和阿霉素加利妥昔单抗 (DA-EPOCH-R) 是否可以改善该人群的预后。布鲁顿酪氨酸激酶抑制剂（BTKi）、BCL-2抑制剂和CXCR4拮抗剂等几种新药以及免疫疗法可能有助于改善CD5 DLBCL患者的预后，但还需要更多的临床探索来确定最佳治疗方案针对这种疾病的策略。© 2024。作者获得 Springer-Verlag GmbH 德国（Springer Nature 旗下公司）的独家许可。

Diffuse large B-cell lymphoma (DLBCL), the most common non-Hodgkin's lymphoma (NHL), is substantially heterogeneous. Approximately 5-10% of DLBCLs express CD5, which makes CD5+ DLBCL a rare subgroup. Different studies have shown that CD5+ DLBCL patients are often older and female and have higher lactate dehydrogenase levels, an Eastern Cooperative Oncology Group (ECOG) performance status > 1, and higher International Prognostic Index (IPI) scores. Moreover, patients often have advanced stage disease with a high incidence of central nervous system (CNS) relapse and bone marrow involvement. CD5+ DLBCL cells are more likely to express MYC, BCL-2, and MUM-1, less likely to express CD10, and most belong to the activated B-cell-like (ABC) subtype. The potential mechanisms underlying the poor prognosis of CD5+ DLBCL patients may be related to CD5-mediated B-cell receptor (BCR)-dependent and -independent pathways. The efficacy of the traditional rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen is unsatisfactory in CD5+ DLBCL patients. Despite supporting evidence from retrospective studies, it is currently unclear whether dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin plus rituximab (DA-EPOCH-R) can improve outcomes in this population. Several new drugs, such as Bruton tyrosine kinase inhibitors (BTKi), BCL-2 inhibitors, and CXCR4 antagonists, as well as immunotherapy, may help to improve the prognosis of CD5+ DLBCL patients, but additional clinical explorations are needed to determine the optimal therapeutic strategy for this disease.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.