早期原发系统间变性大细胞淋巴瘤治疗进展

Treatment advances in early-stage primary systemic anaplastic large cell lymphoma

原发系统间变性大细胞淋巴瘤（ALCL）是一组临床罕见的异质性疾病，早期更为少见。目前缺乏标准的治疗方案，临床多采用类似于B细胞淋巴瘤的治疗方案，回顾性研究中主要的治疗手段为化疗联合局部区域放疗。由于缺乏前瞻性研究，因此对于放疗在其中的治疗意义仍不清楚。此外，合适的化疗方案以及造血干细胞移植在其中的价值尚不明确。文章对早期ALCL的治疗进展进行综述。

Primary systemic anaplastic large cell lymphoma （ALCL） is a subtype of rare and heterogeneous clinical entity, extremely rare in early stage of disease. There is no standard treatment yet. Based on retrospective studies, systematic chemotherapy followed by involved field radiation after chemotherapy is the mainstay of treatment. The role of radiotherapy in the treatment of early stage ALCL remains unclear due to lack of prospective studies. So do the optimal chemotherapy regimens and hematopoietic stem cell transplantation. The paper will review these issues.