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儿童、青少年及年轻成人淋巴母细胞淋巴瘤的诊断与管理

英文原题:Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults.

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Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults.

PubMed 2023/02/16(内容时间) Best Pract Res Clin Haematol Q2 · IF 3.4(JCR 2025)

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中文摘要

淋巴母细胞淋巴瘤(LBL)是儿童、青少年和年轻成人(CAYA)中第二常见的非霍奇金淋巴瘤(NHL),占全部病例的25%–35%。T淋巴母细胞淋巴瘤(T-LBL)占病例的70%–80%,前体B淋巴母细胞淋巴瘤(pB-LBL)占其余20%–25%。采用目前治疗后,儿童LBL患者无事件生存期和总生存期(EFS和OS)均超过80%。治疗方案较为复杂,尤其是纵隔肿瘤体积较大的T-LBL,且具有显著毒性和长期并发症。虽然T-LBL和pB-LBL经一线治疗总体预后良好,但复发/难治性(r/r)疾病患者结局仍极差。本文综述LBL发病机制和生物学的新认识、近期临床结果及未来治疗方向,并讨论改善结局同时降低毒性仍需克服的障碍。

展开英文摘要原文

Lymphoblastic lymphoma (LBL) is the second most common type of non-Hodgkin Lymphoma (NHL) in children, adolescents, and young adults (CAYA), accounting for 25-35% of all cases. T-lymphoblastic lymphoma (T-LBL) comprises 70-80% of cases, while precursor B-lymphoblastic lymphoma (pB-LBL) makes up the remaining 20-25% of cases.

Event-free and overall survival (EFS and OS) for paediatric LBL patients both exceed 80% with current therapies. Treatment regimens, especially in T-LBL with large mediastinal tumours, are complex with significant toxicity and long-term complications. Though prognosis overall is good for T-LBL and pB-LBL with upfront therapy, outcomes for patients with relapsed or refractory (r/r) disease remain dismal.

Here, we review new understanding about the pathogenesis and biology of LBL, recent clinical results and future directions for therapy, and remaining obstacles to improve outcomes while reducing toxicity.

论文信息

作者
Temple WC、Mueller S、Hermiston ML、Burkhardt B
第一作者单位
Paediatric Haematology and Oncology, University of California, San Francisco, USA; Paediatric Allergy, Immunology, and Bone Marrow Transplantation, University of California, San Francisco, USA.United States
通讯作者单位
Paediatric Haematology and Oncology, University of California, San Francisco, USA; Paediatric Allergy, Immunology, and Bone Marrow Transplantation, University of California, San Francisco, USA. Electronic address: Michelle.hermiston@ucsf.edu.United States
文献类型
综述 · 美国 NIH 资助研究 · 非美国政府资助研究
期刊
Best practice & research. Clinical haematology2023 Mar
原文标识
PubMed 36907639 · DOI 10.1016/j.beha.2023.101449