The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China

• DOI: 10.3389/fonc.2022.846573
• Publication Date: 2022-06-06T04:52:53Z
• Authors: Jinyan Xiao; Zihong Cai; Hao Wang; Xuekai Li; Biqi Zhou; Yujie Liu; Ying Wang; Peipei Xu; Li Wang; Depei Wu; Liping Dou; Hongsheng Zhou; Yang Xu
• Keywords: Decitabine; Regimen

Early T-cell precursor (ETP) lymphoblastic leukemia/lymphoma is a high-risk T (T-ALL/LBL) subgroup. We performed real-world multicenter study to explore the clinical characteristics and prognosis of adolescent young adults (AYA) older adult ETP leukemia/lymphoma. A total 103 patients with ETP-ALL/LBL in five centers China between January 2016 February 2021 were included this study. The median age was 29 years (range, 15–70 years). Next-generation sequencing 94 revealed that NOTCH1 (35.1%, 33 cases) most frequently mutated gene, followed by JAK3 (16.0%, 15 cases), PHF6 (13.80%, 13 EZH2 (11.70%, 11 cases). Complete remission (CR) obtained 74.2% (72/97) patients, 6 relapsed/refractory received decitabine combined AAG priming regimen as reinduction therapy CR rate 50%. With follow-up 18 months (0.5–60 months), 2-year overall survival (OS) relapse-free (RFS) rates for entire cohort 54% 57.7%, respectively. Allogeneic stem cell transplantation (allo-SCT) 59.8% (58/97) patients. After landmark analysis at months, OS 77% allo-SCT CR1 25% chemotherapy alone (p < 0.001). multivariate suggested after first course induction independent prognostic factors OS. Collectively, we reported largest AYA ETP-ALL/LBL, found highly invasive had poor long-term prognosis. Allo-SCT could significantly improve patient survival.