Department of Internal Medicine, Bone marrow Transplantation and Cellular Therapy program, King Hussein Cancer Center, Amman, Jordan.
Department of Laboratory sciences, Al-Ahliyya Amman University, Al-Salt, Jordan.
Clin Lymphoma Myeloma Leuk. 2023 Nov;23(11):e411-e419. doi: 10.1016/j.clml.2023.08.007. Epub 2023 Sep 6.
Early T-cell precursor (ETP) acute lymphoblastic leukemia/lymphoma (ALL/LBL) is a newly recognized entity of T-lymphoblastic leukemia/lymphoma. The optimal therapeutic approaches to adult patients are poorly studied.
We compared the outcomes of adult's patents with ETP-ALL/LBL who received frontline chemotherapy regimens with other T-ALL/LBL immunophenotypic subtypes. Patients with ETP-ALL/LBL were identified based on CD1a (-), CD8 (-), CD5 (-) (dim), and positivity for 1 or more stem cell or myeloid antigens.
Sixty-nine patients were included between the years 2010 and 2021 (19 ETP-T-ALL/LBL; 50 non ETP- T-cell ALL/LBL). The median age was 26 year (IQR: 21, 33). Fifty-six patients presented as ALL, while 16 with lymphoblastic lymphoma. Forty-seven patients achieved complete remission, and 43 were alive at last encounter. The complete remission rate in patients with ETP-ALL/LBL was lower than that of non-ETP-ALL/LBL patients (32% vs. 68%; P = .2), and the MRD at end of induction was significantly higher (26% vs. 6.2%, P < .001), and more likely to receive allo-SCT consolidation in CR1 (95% vs. 40%, P < .001). After a median follow-up of survivors of 48 months (range: 32-74 months), the median overall survival for patients with ETP-ALL/LBL was not reached versus 11.5 months for the non-ETP-ALL/LBL patients (P = .014)). Twenty-six patients receive allo-SCT in CR1. There was no significant difference in overall survival (79% vs. 70%; P = .49) between both transplant-cohorts in both groups.
ETP-ALL/LBL represents a high-risk disease subtype of adult ALL. Novel treatment strategies are needed to improve treatment outcomes in this patient's population.
早期 T 细胞前体(ETP)急性淋巴细胞白血病/淋巴瘤(ALL/LBL)是一种新确认的 T 淋巴细胞白血病/淋巴瘤实体。成人患者的最佳治疗方法研究甚少。
我们比较了接受一线化疗方案的成人 ETP-ALL/LBL 患者与其他 T-ALL/LBL 免疫表型亚型患者的结局。基于 CD1a(-)、CD8(-)、CD5(低)和 1 个或多个干细胞或髓样抗原阳性,将 ETP-ALL/LBL 患者识别出来。
2010 年至 2021 年期间纳入了 69 例患者(19 例 ETP-T-ALL/LBL;50 例非 ETP-T 细胞 ALL/LBL)。中位年龄为 26 岁(IQR:21,33)。56 例患者表现为 ALL,16 例为淋巴母细胞淋巴瘤。47 例患者获得完全缓解,43 例在最后一次随访时存活。ETP-ALL/LBL 患者的完全缓解率低于非 ETP-ALL/LBL 患者(32%比 68%;P=0.2),诱导结束时的微小残留病明显更高(26%比 6.2%,P<0.001),且更有可能在 CR1 时接受异体造血干细胞移植巩固(95%比 40%,P<0.001)。在幸存者的中位随访 48 个月(范围:32-74 个月)后,ETP-ALL/LBL 患者的中位总生存期未达到,而非 ETP-ALL/LBL 患者为 11.5 个月(P=0.014)。26 例患者在 CR1 时接受了异体造血干细胞移植。在两组中,CR1 时接受移植的两组患者的总生存率均无显著差异(79%比 70%;P=0.49)。
ETP-ALL/LBL 是成人 ALL 的一种高危疾病亚型。需要新的治疗策略来改善该患者人群的治疗结果。