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套细胞淋巴瘤的现代管理

英文原题:Modern Management of Mantle Cell Lymphoma.

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Modern Management of Mantle Cell Lymphoma.

PubMed 2026/05/07(内容时间) Am Soc Clin Oncol Educ Book

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中文摘要

套细胞淋巴瘤(MCL)是一种生物学和临床上异质性的B细胞恶性肿瘤,预后可变,从惰性、无症状的形式到早期治疗失败的侵袭性亚型不等。当代管理强调风险适应策略,整合患者特征、临床疾病负担和肿瘤生物学。预后工具如MCL国际预后指数(MIPI)及其生物学整合变体(联合MIPI),以及Ki-67增殖、TP53状态和母细胞样形态的评估,有助于指导治疗选择。在年轻、适合的患者中,一线治疗传统上涉及剂量强化的化学免疫治疗,包括高剂量阿糖胞苷和自体干细胞移植(ASCT)。将布鲁顿酪氨酸激酶抑制剂(BTKi),如依鲁替尼,纳入诱导方案已改善生存结果,新出现的证据可能将ASCT的使用限制在高风险亚组。维持治疗,特别是利妥昔单抗,对于持久疾病控制仍至关重要。在老年或不适合移植的患者中,苯达莫司汀-利妥昔单抗仍是骨干治疗,而无化疗组合 incorporating BTKi、BCL2抑制剂和抗CD20抗体提供有效、耐受性良好的替代方案。高风险患者,包括那些有TP53突变的患者,可能受益于靶向三联方案或早期细胞治疗。复发/难治性MCL越来越多地通过共价和非共价BTKi、BCL2抑制剂和T细胞重定向疗法包括CAR-T 细胞疗法和双特异性抗体进行管理。正在进行的试验正在评估最佳序贯和联合策略以改善结局,尤其是在高危和cBTKi暴露患者中。

总体而言,现代MCL管理强调基于生物学风险、功能状态和治疗耐受性的个体化治疗,新型靶向和细胞疗法正在重塑一线和复发治疗格局。

展开英文摘要原文

Mantle cell lymphoma (MCL) is a biologically and clinically heterogeneous B-cell malignancy with variable prognosis, ranging from indolent, asymptomatic forms to aggressive subtypes with early treatment failure. Contemporary management emphasizes risk-adapted strategies that integrate patient characteristics, clinical disease burden, and tumor biology. Prognostic tools such as the MCL International Prognostic Index (MIPI) and its biologically integrated variant (combined MIPI), alongside assessment of Ki-67 proliferation, TP53 status, and blastoid morphology, help guide treatment selection. In younger, fit patients, first-line therapy traditionally involves dose-intensified chemoimmunotherapy with high-dose cytarabine and autologous stem-cell transplantation (ASCT). The incorporation of Bruton tyrosine kinase inhibitors (BTKi), such as ibrutinib, into induction regimens has improved survival outcomes, with emerging evidence that may limit the use of ASCT to high-risk subsets.

Maintenance therapy, particularly rituximab, remains crucial for durable disease control. In older or transplant-ineligible patients, bendamustine-rituximab remains a backbone therapy, with chemotherapy-free combinations incorporating BTKi, BCL2 inhibitors, and anti-CD20 antibodies offering effective, well-tolerated alternatives. High-risk patients, including those with TP53 mutations, may benefit from targeted triplet regimens or early cellular therapies.

Relapsed/refractory MCL is increasingly managed with covalent and noncovalent BTKi, BCL2 inhibitors, and T-cell-redirecting therapies including chimeric antigen receptor T-cell therapy and bispecific antibodies. Ongoing trials are evaluating optimal sequencing and combination strategies to improve outcomes, particularly in high-risk and cBTKi-exposed patients.

Overall, modern MCL management emphasizes individualized therapy based on biological risk, functional status, and treatment tolerability, with novel targeted and cellular approaches reshaping the frontline and relapsed treatment landscape.

论文信息

作者
Tix T、Kumar A、Eyre TA、Dreyling M
单位
Department of Medicine III, LMU Hospital, Munich, Germany.Germany
文献类型
综述
期刊
American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting2026 Jun
原文标识
PubMed 42096665 · DOI 10.1200/EDBK-26-517468