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儿童间变性大细胞淋巴瘤的进展与更新

英文原题:Advances and updates in pediatric anaplastic large cell lymphoma.

PubMed 2025/10/14(内容时间) Blood Adv Q1 · IF 7.7(JCR 2025)

研究概要

尽管采用了密集的多药化疗方案,治疗失败率仍高达25%至30%。

中文摘要

间变性大细胞淋巴瘤(ALCL)是一种罕见的成熟T细胞非霍奇金淋巴瘤。在儿科患者中,大多数病例为间变性淋巴瘤激酶(ALK)阳性。尽管采用了强化多药联合化疗方案,治疗失败率仍维持在25%至30%。近年来靶向治疗的进展,尤其是ALK抑制剂和抗CD30抗体药物偶联物brentuximab vedotin,在复发和难治性情况下已显示出显著活性。微小播散性疾病(MDD)和微小残留病(MRD)的分子检测可改善预后分层。对于复发或难治性疾病的患者,靶向治疗增加了治疗选择,但仍需开展更多工作以确定该组患者的最佳治疗方案、疗程以及是否需要造血干细胞移植。免疫治疗如检查点抑制剂或CAR-T 细胞疗法提供了额外的治疗选择。将靶向治疗和MDD/MRD评估纳入临床试验,可能显著改善儿童和青少年ALCL患者的预后。

展开英文摘要原文

Anaplastic large cell lymphoma (ALCL) is a rare form of mature T-cell non-Hodgkin lymphoma. In pediatric patients, most cases are anaplastic lymphoma kinase (ALK) positive. Despite intensive multiagent chemotherapy regimens, treatment failure rates remain at 25% to 30%. Recent advancements in targeted therapies, notably ALK inhibitors and the anti-CD30 antibody-drug conjugate brentuximab vedotin have demonstrated substantial activity in relapsed and refractory settings. Molecular detection of minimal disseminated disease (MDD) and minimal residual disease (MRD) offers improved prognostic stratification. For patients with relapsed or refractory disease, targeted therapies have increased treatment options, but more work needs to be done to define optimal treatment regimens, duration, and need for hematopoietic stem cell transplantation in this group. Immune therapies such as checkpoint inhibitors or chimeric antigen receptor T-cell therapy provide additional therapeutic options. Incorporating targeted therapies and MDD/MRD assessments into clinical trials could significantly improve outcomes for pediatric and adolescent patients with ALCL.

论文信息

作者
Marks LJ、Lowe E、Kamdar K
第一作者单位
Division of Pediatric Hematology, Oncology, Stem Cell Transplantation, and Regenerative Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA.United States
通讯作者单位
Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine, Houston, TX.United States
文献类型
综述
期刊
Blood advances2025 Oct 14
原文标识
PubMed 40690755 · DOI 10.1182/bloodadvances.2025015935