Division of Hematology, University of Washington, Seattle, WA.
Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA.
Blood Adv. 2021 Feb 9;5(3):640-648. doi: 10.1182/bloodadvances.2020001581.
Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (ALK- ALCL) is an aggressive neoplasm of T-cell/null-cell lineage. The T-Cell Project is a global prospective cohort study that consecutively enrolled patients newly diagnosed with peripheral T-cell lymphoma, registered through a centralized computer database between September 2006 and February 2018. Of 1553 validated cases from 74 sites in 13 countries worldwide, 235 were reported as ALK- ALCL. The median age at diagnosis was 54 years (range, 18-89 years), with a male predominance (62%). Stage III to IV disease was identified in 71% of patients, bulky disease and bone marrow involvement were uncommon, and 66% of patients presented with a low (0-1) International Prognostic Index score. Of all treated patients, 85% received multiagent initial chemotherapy, and 8% were consolidated with autologous hematopoietic cell transplantation. The initial overall and complete response rates were 77% and 63%, respectively. After a median follow-up of 52 months (95% confidence interval [CI], 41-63), the median progression-free survival (PFS) and overall survival (OS) were 41 months (95% CI, 17-62) and 55 months (95% CI, 36-75), respectively. The 3- and 5-year PFS rates were 52% and 43%, and the 3- and 5-year OS rates were 60% and 49%. Treatments containing both anthracycline and etoposide were associated with superior OS (P = .05) but not PFS (P = .18). In this large prospective cohort study, outcomes comparable to those previously reported in the retrospective International Peripheral T-Cell Lymphoma Project were observed. The study underscores the need for introducing novel platforms for ALK- ALCL and establishes a benchmark for future clinical trials. This trial was registered at www.clinicaltrials.gov as #NCT01142674.
间变性大细胞淋巴瘤（ALK- ALCL）是一种侵袭性的 T 细胞/无细胞谱系肿瘤。T 细胞项目是一项全球性的前瞻性队列研究，该研究连续纳入了 2006 年 9 月至 2018 年 2 月期间通过集中计算机数据库登记的外周 T 细胞淋巴瘤新诊断患者。在全球 13 个国家的 74 个地点的 1553 例经证实的病例中，有 235 例报告为 ALK- ALCL。诊断时的中位年龄为 54 岁（范围，18-89 岁），男性居多（62%）。71%的患者存在 III 期至 IV 期疾病，大肿块和骨髓累及不常见，66%的患者国际预后指数评分较低（0-1）。所有接受治疗的患者中，85%接受了多药初始化疗，8%接受了自体造血细胞移植巩固治疗。初始总缓解率和完全缓解率分别为 77%和 63%。中位随访 52 个月（95%置信区间[CI]，41-63）后，中位无进展生存期（PFS）和总生存期（OS）分别为 41 个月（95%CI，17-62）和 55 个月（95%CI，36-75）。3 年和 5 年 PFS 率分别为 52%和 43%，3 年和 5 年 OS 率分别为 60%和 49%。包含蒽环类药物和依托泊苷的治疗方案与更好的 OS 相关（P =.05），但与 PFS 无关（P =.18）。在这项大型前瞻性队列研究中，观察到的结果与先前在回顾性国际外周 T 细胞淋巴瘤项目中报告的结果相当。该研究强调需要为 ALK- ALCL 引入新的治疗平台，并为未来的临床试验建立基准。该试验在 www.clinicaltrials.gov 上注册为 #NCT01142674。
