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EHA-EU MCL 网络套细胞淋巴瘤诊断与治疗指南

英文原题:EHA-EU MCL network guidelines for diagnosis and treatment of mantle cell lymphoma.

查看英文原题

EHA-EU MCL network guidelines for diagnosis and treatment of mantle cell lymphoma.

PubMed 2025/10/22(内容时间) Hemasphere Q1 · IF 11.3(JCR 2025)

研究概要

套细胞淋巴瘤 (MCL) 是一种相对罕见的 B 细胞淋巴瘤亚型,男性发病率较高,诊断时中位年龄为 70 岁。

中文摘要

套细胞淋巴瘤(MCL)是较罕见的B细胞淋巴瘤亚型,男性发病率较高,诊断时年龄中位数为70岁。MCL临床表现多样,可从惰性病程到极具侵袭性,这与增殖速率和TP53突变等生物学危险因素有关。患者通常确诊时已为播散性疾病,因此需要全身治疗。免疫化疗历来是主要治疗方式,但近期数据表明,加入新型药物,尤其是共价布鲁顿酪氨酸激酶抑制剂(cBTKi),可能显著改善年轻和老年患者的结局,不过其能否实现治愈仍有待证实。对于老年患者,利妥昔单抗联合苯达莫司汀等免疫化疗仍是标准治疗,但利妥昔单抗联合cBTKi等非化疗方案可能对其构成挑战。复发或难治性患者的治疗选择正在快速发展,包括CAR-T细胞疗法、新型BTK靶向药物、BCL2抑制剂和T细胞接合抗体。本临床实践指南提出MCL诊断、分期、治疗和随访的现行循证建议。

展开英文摘要原文

Mantle cell lymphoma (MCL) is a relatively rare B-cell lymphoma subtype, with a higher incidence among males and a median age of 70 years at diagnosis. MCL is characterized by clinically diverse behavior, from indolent disease to extremely aggressive, related to the presence of biological risk factors such as proliferation rate and TP53 mutations. Most often, patients present with disseminated disease, necessitating systemic treatment. Immunochemotherapy has historically been the mainstay of treatment, but recent data indicate that addition of novel agents, especially covalent Bruton tyrosine kinase inhibitors (cBTKi), may substantially improve outcome in younger and older patients, although a curative approach remains to be shown. In elderly patients, the standard of care is still immuno-chemotherapy such as rituximab-bendamustine, although this may be challenged by non-chemotherapeutic options, such as rituximab plus cBTKi. For patients with relapsed or refractory disease, treatment options are developing rapidly, including CAR-T cell therapy, novel BTK targeting agents, BCL2 inhibitors, and T-cell engagers. In this clinical practice guideline, we present current evidence-based recommendations for diagnosis, staging, treatment, and follow-up of MCL.

论文信息

作者
Jerkeman M、Aurer I、Campo E、Cheah CY、Clark J、Doorduijn J、Eyre TA、Fehr M
第一作者单位
Department of Oncology Skåne University Hospital Lund Sweden.Sweden
通讯作者单位
Department of Medicine III LMU University Hospital Munich.Germany
期刊
HemaSphere2025 Oct
原文标识
PubMed 41132246 · DOI 10.1002/hem3.70233