儿童间变性大细胞淋巴瘤（ALCL）是一种临床上少见的非霍奇金淋巴瘤。形态上存在着一种标志的 “hallmark”细胞；细胞免疫学上CD30（Ki-1）表达强阳性；标志性的染色体异常为t（2； 5）（ p23；q35）。90%以上的ALCL 患儿间变淋巴瘤激酶（ALK）阳性。临床诊断时多为疾病晚期（Ⅲ~Ⅳ期），并常伴有B症状（发热、盗汗、消瘦等）。 一线治 疗方案仍以化疗为主，但不管采用何种方案，其3~5年无事件生存率（EFS）都在65%~75%之间。高危及复发难治ALCL 可每周单药使用长春花碱和/或重新化疗使其完全缓解（CR）后再实施造血干细胞移植治疗。新药如CD30单克隆抗体和 ALK蛋白抑制剂、针对CD30的嵌合抗原受体T细胞等治疗选择将来有望应用于儿童ALCL。

Anaplastic large cell lymphoma (ALCL) is a clinically rare non-Hodgkin’s lymphoma which is characterized by malignant “hallmark cells” and strong expression of CD30 in membrane and golgi patterns in almost all cases. The most frequent chromosomal translocation is t (2;5)(p23;q35). ALCL in children is nearly universally anaplastic lymphoma kinase (ALK) positive and commonly present with advanced systemic disease. Many different treatment strategies have been utilized with similar event free survival rates of 65%~75%. High-risk and recurrent/refractory ALCL can be treated by vinblastine weekly or hematopoietic stem cell transplantation. Novel therapies such as CD30 targeted treatment and ALK inhibitors may soon radically change the treatment paradigm for this disease. This review will provide an overview of the biological characteristics, clinical features, treatment and prognostic factors for children with ALCL.