Real world outcome of B ALL with t (1; 19) (q23; p13)/TCF3::PBX1 in adolescents and adults treated with intensive regimes

IF 2.1 4区医学 Q3 HEMATOLOGY Leukemia research Pub Date : 2024-04-18 DOI:10.1016/j.leukres.2024.107506

Tribikram Panda, Sujay Rainchwar, Reema Singh, Aakanksha Singh, Mayank Soni, Disha Kakkar, KR Jegan, Reshmi Harikumar Pillai, Roy J. Palatty, Karuna Jha, Rayaz Ahmed, Rohan Halder, Narender Tejwani, Devasis Panda, Dinesh Bhurani, Narendra Agrawal

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Abstract

Significant heterogeneity has been reported in outcome of Acute lymphoblastic leukemia with t(1;19)(q23;p13)/TCF3::PBX1 in adolescents and adults leading to a lack of consensus on precise risk stratification. We evaluated clinical outcome of 17 adult ALL cases (≥15 years) with this genotype treated on intensive regimes.13/17 received COG0232 and 4/17 cases received UK-ALL protocol. All achieved CR (100%) with above treatment. End of induction MRD was evaluated in 14/17 cases of which 11 (78.5%) achieved MRD negativity. Total nine patients relapsed (7 marrows, 2 CNS). Overall survival at 2 years was 53.3%. The 2 year estimated PFS was 42.9%. The 2 years CIR was 54.2%. Adults with this genotype perform poorly despite early favorable response. Incorporation of novel immunotherapies and prompt HSCT should be strongly considered with this genotype. Targeted NGS panels for additional genetic aberrations can further help in risk stratifying and guiding therapy for this genotype.

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青少年和成人中接受强化治疗的 t (1; 19) (q23; p13)/TCF3::PBX1 B ALL 的实际疗效

有报道称，青少年和成人急性淋巴细胞白血病（t(1;19)(q23;p13)/TCF3::PBX1）患者的预后存在显著异质性，导致人们对精确的风险分层缺乏共识。我们评估了17例成人ALL病例（≥15岁）的临床疗效，其中13/17接受了COG0232治疗，4/17接受了UK-ALL方案治疗。13/17例接受了COG0232治疗，4/17例接受了UK-ALL方案治疗。对 14/17 例患者的诱导末期 MRD 进行了评估，其中 11 例（78.5%）达到 MRD 阴性。共有9例患者复发（7例骨髓，2例中枢神经系统）。2年总生存率为53.3%。估计两年的 PFS 为 42.9%。2年的CIR为54.2%。尽管早期反应良好，但这种基因型的成人表现不佳。对于这种基因型的患者，应积极考虑采用新型免疫疗法和及时进行造血干细胞移植。针对其他基因畸变的有针对性的 NGS 面板可进一步帮助对该基因型进行风险分层和指导治疗。

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来源期刊

Leukemia research 医学-血液学

CiteScore

4.00

自引率

3.70%

发文量

259

审稿时长

1 months

期刊介绍： Leukemia Research an international journal which brings comprehensive and current information to all health care professionals involved in basic and applied clinical research in hematological malignancies. The editors encourage the submission of articles relevant to hematological malignancies. The Journal scope includes reporting studies of cellular and molecular biology, genetics, immunology, epidemiology, clinical evaluation, and therapy of these diseases.