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早期 T 细胞前体细胞白血病:一种高风险的急性淋巴细胞白血病亚型,约旦单中心经验。

Early T-Cell Precursor Leukemia: A High-Risk Subtype of Acute Lymphoblastic Leukemia, Single Center Experience in Jordan.

机构信息

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.

出版信息

Clin Lymphoma Myeloma Leuk. 2023 Nov;23(11):e411-e419. doi: 10.1016/j.clml.2023.08.007. Epub 2023 Sep 6.

Abstract

BACKGROUND AND OBJECTIVES

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.

PATIENTS AND METHODS

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.

RESULTS

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.

CONCLUSION

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.

摘要

背景与目的

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。

方法

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。

结果

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)。

结论

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。

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