ALK阳性的间变性大细胞淋巴瘤（ALCL）约占成熟T细胞淋巴瘤的6-7％。该亚型包含ALK基因在染色体2上的易位与其他几个基因之间的易位，共同形成一个癌基因。最常见的易位是t（2; 5），它将ALK与NPM1结合在一起。这种淋巴瘤的中位年龄为34岁，男性较常见，在大多数患者的诊断时处于晚期。ALK阳性ALCL是周围T细胞淋巴瘤中最治愈的一种。CHOP方案是最常用的，但用文替麦滴定替代长春新碱（BV-CHP）和加入顺铂（CHOEP）可以改善疗效，其中BV-CHP更受欢迎。拯救疗法包括异基因或自体骨髓移植，BV（如果未用作主要治疗的一部分）和ALK抑制剂。后者非常有效，很可能会纳入到主要治疗中。 版权：© 2023 Armitage JO et al.

ALK-positive anaplastic large cell lymphoma (ALCL) represents approximately 6-7% of the mature T-cell lymphomas. This subtype contains a translocation between the ALK gene on chromosome 2 and one of several other genes that together form an oncogene. The most frequent translocation is t(2;5) which combines ALK with NPM1. This lymphoma has a median age of 34 years, is more common in males, and is in advanced stage at the time of diagnosis in most patients. ALK-positive ALCL is the most curable of the peripheral T-cell lymphomas. The CHOP regimen has been most frequently used, but results are improved with the substitution of brentuximab vedotin for vincristine (BV-CHP) and the addition of etoposide (CHOEP), with BV-CHP being favored. Salvage therapies include allogeneic or autologous bone marrow transplantation, BV, if not used as part of the primary therapy, and ALK inhibitors. The latter are very active and likely to be incorporated into the primary therapy.Copyright: © 2023 Armitage JO et al.