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.

淋巴母细胞淋巴瘤 (LBL) 是儿童、青少年和年轻人 (CAYA) 中第二常见的非霍奇金淋巴瘤 (NHL) 类型，占所有病例的 25-35%。T 淋巴母细胞淋巴瘤 (T-LBL) 占病例的 70-80%，而前体 B 淋巴母细胞淋巴瘤 (pB-LBL) 占其余 20-25% 的病例。使用当前疗法，儿科 LBL 患者的无事件生存率和总生存率（EFS 和 OS）均超过 80%。治疗方案，特别是在伴有大纵隔肿瘤的 T-LBL 中，治疗方案复杂，具有显着的毒性和长期并发症。尽管通过前期治疗，T-LBL 和 pB-LBL 的总体预后良好，但复发或难治性 (r/r) 疾病患者的预后仍然不佳。在这里，我们回顾了对 LBL 发病机制和生物学的新认识，