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病例报告:伴有罕见 ABL1 重排的 T 细胞急性淋巴细胞白血病对 TKI 治疗的临床反应

英文原题:Case Report: Clinical response to TKI therapy in T-cell acute lymphoblastic leukemia with rare ABL1 rearrangement.

PubMed 2026/09/10(内容时间) Front Oncol Q2 · IF 3.4(JCR 2025)

研究概要

本文报告了迄今为止第二例携带ZBTB16::ABL1融合基因的T细胞急性淋巴细胞白血病接受TKI治疗的病例。

中文摘要

背景:伴ABL1重排的T细胞急性淋巴细胞白血病(T-ALL)是一种极为罕见的疾病,其临床意义尚未被准确定义。我们报道一例携带ABL1重排的儿童T细胞急性淋巴细胞白血病患者的临床特征及对酪氨酸激酶抑制剂(TKI)治疗的反应,为该亚型的临床管理提供参考。病例报告:一名3岁男孩,患有T-ALL,RNA-seq检出ZBTB16::ABL1融合,对诱导化疗反应差,D33未达缓解。在CAM化疗的同时加用达沙替尼(75 mg/m²/天)。至D90时,达到形态学CR且流式MRD阴性,但qPCR仍为阳性,为0.08%。在Block III巩固治疗期间发生孤立性CNS复发。

展开英文摘要原文

BACKGROUND: T-cell acute lymphoblastic leukemia (T-ALL) with ABL1 rearrangement is an extremely rare disease, and its clinical significance has not been precisely defined. We report the clinical characteristics of a pediatric patient with T-cell acute lymphoblastic leukemia harboring ABL1 rearrangement and the treatment response to tyrosine kinase inhibitor (TKI) therapy, providing a reference for clinical management with this subtype. CASE REPORTS: A 3-year-old boy with T-ALL and ZBTB16::ABL1 fusion (RNA-seq) showed poor response to induction chemotherapy, failing remission at D33. Dasatinib (75 mg/m 2 /day) was added concurrently with CAM chemotherapy. At D90, morphological CR and flow-MRD negativity were achieved, though qPCR remained positive at 0.08%. Isolated CNS relapse occurred during Block III consolidation. Subsequent management included autologous CD7 CAR T-cell therapy and allogeneic HSCT; post-transplant evaluation confirmed sustained leukemia remission. However, the patient developed HHV-6 encephalitis and adenovirus hepatitis with hepatic failure; life-sustaining support was withdrawn at the family's request. The patient was discharged and lost to follow-up. CONCLUSION: This paper presents the second reported case to date of TKI treatment for T-cell acute lymphoblastic leukemia harboring the ZBTB16::ABL1 fusion. Following a review of the relevant literature on ABL1 -rearranged T-ALL, we conclude that the accumulation of additional cases is essential to better characterize this rare molecular subtype and to guide evidence-based treatment decisions.

论文信息

作者
Zhong M、He W、Zeng L、Zhang J
单位
Department of Paediatric Hematology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, Guangdong, China.China
文献类型
病例报告
期刊
Frontiers in oncology2026
原文标识
PubMed 42787271 · DOI 10.3389/fonc.2026.1928646