Chiattone Carlos, Civallero Monica, Fischer Thais, Miranda Eliana, Manni Martina, Zing Natalia P C, Pileri Stefano A, Montoto Silvia, Horwitz Steven M, Cabrera Maria Elena, De Souza Carmino A, Nagler Arnon, Luminari Stefano, Ferreri Andrés J M, Carson Kenneth R, Re Alessandro, Rigacci Luigi, Nassi Luca, Stepanishyna Yana, Federico Massimo, Inghirami Giorgio
Santa Casa Medical School of Sao Paulo, Samaritano Hospital, São Paulo, Brazil.
Department of CHIMOMO, University of Modena and Reggio Emilia, Modena, Italy.
Hematol Oncol. 2022 Dec;40(5):953-961. doi: 10.1002/hon.3074. Epub 2022 Sep 15.
The T-cell Lymphoma Project is an international registry prospective study that enrolled patients with newly diagnosed peripheral T-cell and NK-cell lymphomas (PTCL). The main objective was to define the clinical features and outcomes, establishing a robust benchmark for future clinical trials. Seventy-four institutions from 14 countries in North America, South America, Europe, and Asia collected data on patients diagnosed and treated at their respective centers between September 2006 and February 2018. Among 1553 PTCL patients, 131 (8.4% of the total cohort) were confirmed to have anaplastic large cell lymphoma - kinase positive (ALCL, ALK+). The median age of the patients was 39 years (18-84). Sixty-five patients (66%) had advanced-stage disease, although majority (45 patients, 54%) had a low-risk International Prognostic Index (IPI) score (0-1). Of 97 patients treated with chemotherapy, 97% received anthracycline-containing regimens. The overall response rate was 81%, with 69 patients (70%) achieving complete remission. Estimated OS and PFS at 3 years were 77% (95% CI: 54%-99%) and 68% (95% CI: 46%-90%), respectively, and at 5 years were very similar, 77% of OS (95% CI: 62%-92%) and 64% of PFS (95% CI: 34%-94%). Multivariate analysis for PFS showed advanced stage (hazard ratios [HR]: 4.72, 95% CI: 1.43-23.9, p = 0.015), elevated lactate dehidrogenade (LDH) (HR 4.85; 95% CI: 1.73-13.60, p = 0.001), and Eastern Cooperative Oncology Group Performance Status scale (ECOG-PS) ≥2 (HR: 5.25; 95% CI: 1.68-16.4, p = 0.024). For OS, elevated LDH (HR: 3.77; 95% CI: 1.98-14.17, p = 0.014) and ECOG-PS ≥2 (HR: 4.59; 95% CI: 1.46-14.39, p = 0.004) were identified. In summary, although the outcome of ALK+ ALCL is superior to that of other PTCLs, it remains sufficiently favorable, given the young median age of the patients. Our results confirm the usefulness of both IPI and Prognostic Index for T-cell Lymphoma (PIT) in identifying groups of patients with different outcomes. Clinical Trials ID: NCT01142674.
T细胞淋巴瘤项目是一项国际注册前瞻性研究，纳入了新诊断的外周T细胞和NK细胞淋巴瘤（PTCL）患者。主要目标是明确临床特征和预后，为未来的临床试验建立一个可靠的基准。来自北美、南美、欧洲和亚洲14个国家的74家机构收集了2006年9月至2018年2月期间在各自中心诊断和治疗的患者的数据。在1553例PTCL患者中，131例（占总队列的8.4%）被确诊为间变性大细胞淋巴瘤激酶阳性（ALCL，ALK+）。患者的中位年龄为39岁（18 - 84岁）。65例患者（66%）患有晚期疾病，尽管大多数（45例，54%）的国际预后指数（IPI）评分较低（0 - 1）。在97例接受化疗的患者中，97%接受了含蒽环类药物的方案。总体缓解率为81%，69例患者（70%）实现完全缓解。3年时的总生存期（OS）和无进展生存期（PFS）估计分别为77%（95%置信区间：54% - 99%）和68%（95%置信区间：46% - 90%），5年时非常相似，OS为77%（95%置信区间：62% - 92%），PFS为64%（95%置信区间：34% - 94%）。PFS的多因素分析显示晚期（风险比[HR]：4.72，95%置信区间：1.43 - 23.9，p = 0.015）、乳酸脱氢酶（LDH）升高（HR 4.85；95%置信区间：1.73 - 13.60，p = 0.001）以及东部肿瘤协作组体能状态量表（ECOG - PS）≥2（HR：5.25；95%置信区间：1.68 - 16.4，p = 0.024）。对于OS，LDH升高（HR：3.77；95%置信区间：1.98 - 14.17，p = 0.014）和ECOG - PS≥2（HR：4.59；95%置信区间：1.46 - 14.39，p = 0.004）被确定为相关因素。总之，尽管ALK+ ALCL的预后优于其他PTCL，但考虑到患者的中位年龄较轻，其预后仍然足够良好。我们的结果证实了IPI和T细胞淋巴瘤预后指数（PIT）在识别不同预后患者群体方面的有用性。临床试验编号：NCT01142674。
