Xiao Jinyan, Cai Zihong, Wang Hao, Li Xuekai, Zhou Biqi, Liu Yujie, Wang Ying, Xu Peipei, Wang Li, Wu Depei, Dou Liping, Zhou Hongsheng, Xu Yang
Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Suzhou, China.
Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Front Oncol. 2022 Jun 6;12:846573. doi: 10.3389/fonc.2022.846573. eCollection 2022.
Early T-cell precursor (ETP) lymphoblastic leukemia/lymphoma is a high-risk T lymphoblastic leukemia/lymphoma (T-ALL/LBL) subgroup. We performed a real-world multicenter study to explore the clinical characteristics and prognosis of adolescent and young adults (AYA) and older adult ETP leukemia/lymphoma. A total of 103 patients with ETP-ALL/LBL in five centers in China between January 2016 and February 2021 were included in this study. The median age was 29 years (range, 15-70 years). Next-generation sequencing was performed in 94 patients and revealed that NOTCH1 (35.1%, 33 cases) was the most frequently mutated gene, followed by JAK3 (16.0%, 15 cases), PHF6 (13.80%, 13 cases) and EZH2 (11.70%, 11 cases). Complete remission (CR) was obtained in 74.2% (72/97) of patients, and 6 relapsed/refractory patients received a decitabine combined with AAG priming regimen as reinduction therapy with a CR rate of 50%. With a median follow-up of 18 months (0.5-60 months), the 2-year overall survival (OS) and relapse-free survival (RFS) rates for the entire cohort were 54% and 57.7%, respectively. Allogeneic stem cell transplantation (allo-SCT) was performed in 59.8% (58/97) of patients. After landmark analysis at 6 months, the 2-year OS rates was 77% of patients with allo-SCT at CR1 and 25% of patients with chemotherapy alone (p < 0.001). A multivariate analysis suggested that allo-SCT and CR after the first course induction were independent prognostic factors for OS. Collectively, we reported the largest cohort study with AYA and older adult ETP-ALL/LBL, and we found that ETP-ALL/LBL was highly invasive and had a poor long-term prognosis. Allo-SCT could significantly improve ETP-ALL/LBL patient survival.
早期T细胞前体（ETP）淋巴细胞白血病/淋巴瘤是高危T淋巴细胞白血病/淋巴瘤（T-ALL/LBL）亚组。我们开展了一项真实世界多中心研究，以探索青少年和青年（AYA）及老年ETP白血病/淋巴瘤的临床特征和预后。2016年1月至2021年2月期间，中国五个中心共103例ETP-ALL/LBL患者纳入本研究。中位年龄为29岁（范围15 - 70岁）。94例患者进行了二代测序，结果显示NOTCH1（35.1%，33例）是最常发生突变的基因，其次是JAK3（16.0%，15例）、PHF6（13.80%，13例）和EZH2（11.70%，11例）。74.2%（72/97）的患者获得完全缓解（CR），6例复发/难治性患者接受地西他滨联合AAG预激方案作为再诱导治疗，CR率为50%。中位随访18个月（0.5 - 60个月），整个队列的2年总生存（OS）率和无复发生存（RFS）率分别为54%和57.7%。59.8%（58/97）的患者接受了异基因造血干细胞移植（allo-SCT）。在6个月进行标志性分析后，CR1期接受allo-SCT患者的2年OS率为77%，单纯化疗患者为25%（p < 0.001）。多因素分析表明，allo-SCT和首次诱导疗程后的CR是OS的独立预后因素。总体而言，我们报告了关于AYA及老年ETP-ALL/LBL的最大队列研究，并且我们发现ETP-ALL/LBL具有高度侵袭性且长期预后较差。allo-SCT可显著改善ETP-ALL/LBL患者的生存。
