Characteristics and outcome of pediatric mixed-phenotype acute leukemia treated with EORTC 58951 protocol: An observational study in Tunisia.

IF 1.3 4区医学 Q3 PEDIATRICS Archives De Pediatrie Pub Date : 2025-03-19 DOI:10.1016/j.arcped.2025.01.001

Ameni Yahia, Marwa Bahri, Yosr Ben Abdennebi, Emna Azza, Fatma Ben Lakhel, Sarra Fekih, Wijden El Borji, Ons Ghali, Emna Gouider, Wiem Ayed, Ahlem Amouri, Lamia Aissaoui

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Abstract

Background: Mixed phenotype acute leukemia (MPAL), also known as biphenotypic acute leukemia (BAL), is an uncommon subgroup of leukemia that exhibits features of both lymphoid and myeloid lineages.

Objective: This study aims to analyze the clinical and biological features of MPAL and to evaluate the therapeutic approaches in children diagnosed with MPAL.

Methods and settings: It was a retrospective study that included children (age<18 years old) diagnosed with MPAL, based on the European Group for Immunological Characterization of Leukemia or the 2008/2016 WHO criteria, in the pediatric hematology department of Aziza Othmana Hospital in Tunisia, from 2006 to 2022.

Results: Of 639 patients with acute leukemia, 10 (1.5%) were diagnosed with MPAL (10 of 639). The median age at diagnosis was 9 years old (range, 4-18 years) with a gender ratio of 1.5. The median initial leukocyte count was 28.3×10⁹/L (range, 1.6-143×10⁹/L). None of the patients had central nervous system involvement. Four patients (40%) had a T/Myeloid phenotype and 6 patients (60%) had a B/Myeloid phenotype. Cytogenetic abnormalities were seen in 7 cases (70%). The BCR-ABL fusion gene was detected in 2 patients (20%). None of the patients had a KMT2A rearrangement. All patients initially received acute lymphoblastic leukemia (ALL) chemotherapy using the EORTC 58951 protocol. Within these patients, one patient (10%) died during the induction phase and 9 (90%) achieved morphologic complete remission at the end of induction. Only one patient underwent allogeneic hematopoietic stem cell transplantation. Treatment-related mortality was 20% (2 cases). The median follow-up time was 38 months (1-202 months). The 3-year event-free and the 3-year overall survival rates for the entire group were 60%.

Conclusion: MPAL is rare and complex, with heterogeneous clinical and biological features. A literature review suggests that ALL chemotherapy is better for achieving a favorable prognosis than AML regimens.

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背景：混合表型急性白血病（MPAL）又称双表型急性白血病（BAL），是一种不常见的白血病亚群，同时表现出淋巴系和髓系的特征：本研究旨在分析MPAL的临床和生物学特征，并评估确诊为MPAL的儿童的治疗方法：这是一项回顾性研究，研究对象包括儿童（年龄为6-12岁）：在639例急性白血病患者中，10例（1.5%）被诊断为MPAL（639例中的10例）。诊断时的中位年龄为 9 岁（4-18 岁），性别比例为 1.5。初始白细胞计数中位数为 28.3×10⁹/L（范围为 1.6-143×10⁹/L）。所有患者均未累及中枢神经系统。4名患者（40%）为T/髓系表型，6名患者（60%）为B/髓系表型。7例患者（70%）出现细胞遗传学异常。2例患者（20%）检测到BCR-ABL融合基因。没有一名患者出现KMT2A重排。所有患者最初都按照 EORTC 58951 方案接受了急性淋巴细胞白血病（ALL）化疗。在这些患者中，1名患者（10%）在诱导阶段死亡，9名患者（90%）在诱导结束时获得了形态学上的完全缓解。只有一名患者接受了异基因造血干细胞移植。治疗相关死亡率为20%（2例）。中位随访时间为38个月（1-202个月）。整组患者的3年无事件生存率和3年总生存率均为60%：MPAL罕见且复杂，具有不同的临床和生物学特征。文献综述表明，与急性髓细胞性白血病方案相比，ALL化疗方案更有利于获得良好的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Archives De Pediatrie 医学-小儿科

CiteScore

2.80

自引率

5.60%

发文量

106

审稿时长

24.1 weeks

期刊介绍： Archives de Pédiatrie publishes in English original Research papers, Review articles, Short communications, Practice guidelines, Editorials and Letters in all fields relevant to pediatrics. Eight issues of Archives de Pédiatrie are released annually, as well as supplementary and special editions to complete these regular issues. All manuscripts submitted to the journal are subjected to peer review by international experts, and must: Be written in excellent English, clear and easy to understand, precise and concise; Bring new, interesting, valid information - and improve clinical care or guide future research; Be solely the work of the author(s) stated; Not have been previously published elsewhere and not be under consideration by another journal; Be in accordance with the journal''s Guide for Authors'' instructions: manuscripts that fail to comply with these rules may be returned to the authors without being reviewed. Under no circumstances does the journal guarantee publication before the editorial board makes its final decision. Archives de Pédiatrie is the official publication of the French Society of Pediatrics.