Department of Pediatric Oncology, Regional Cancer Centre, Thiruvananthapuram, India.
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, India.
Pediatr Hematol Oncol. 2021 May;38(4):358-366. doi: 10.1080/08880018.2020.1871453. Epub 2021 Feb 26.
There is limited data regarding pediatric mixed phenotype acute leukemia (MPAL) and there is no global consensus on its management yet. In this retrospective study, we analyzed the outcomes of children diagnosed with MPAL at our institute. This study included children ≤ 14 years with MPAL who presented to a tertiary cancer center in India from January 1st 2009 to December 31st 2015. Over a seven-year period, 1390 patients with leukemia presented to our institute of which 22 patients (1.5%) had MPAL. Sixteen patients (72.7%) had B/myeloid leukemia, while 4 (18.1%) and 2 (9%) patients had T/myeloid and B/T leukemia respectively. Twenty-one patients were treated with a modified BFM ALL 95 protocol. 76.1% (n = 16) of patients had a good prednisolone response (GPR) on day 8 and end-of-induction (EOI) marrow was in remission in 90.5% (n = 19). A poor prednisolone response (PPR) on day 8 correlated with an inferior relapse-free survival (25% vs 79.5%, P=.025). The 4-year event-free survival (EFS) and overall survival (OS) for the entire group was 60.8% and 64.9% respectively while the EFS for patients who had a GPR and remission at the EOI (n = 15) was 80% as compared to 16.7% in patients with PPR or induction failure. Lymphoid directed chemotherapy is seen to have good survival outcomes in pediatric MPAL. However, a PPR on day 8 or a positive EOI marrow may be an indication for more aggressive treatment.
关于儿科混合表型急性白血病（MPAL）的数据有限，目前尚未达成全球共识。在这项回顾性研究中，我们分析了我院诊断为 MPAL 的儿童的结局。本研究纳入了 2009 年 1 月 1 日至 2015 年 12 月 31 日期间在印度一家三级癌症中心就诊的≤14 岁的 MPAL 患儿。在 7 年期间，共有 1390 例白血病患儿在我院就诊，其中 22 例（1.5%）为 MPAL。16 例（72.7%）患儿为 B/髓性白血病，4 例（18.1%）和 2 例（9%）患儿分别为 T/髓性和 B/T 白血病。21 例患者接受改良 BFM ALL 95 方案治疗。76.1%（n=16）的患者在第 8 天泼尼松反应良好（GPR），诱导结束时（EOI）骨髓缓解率为 90.5%（n=19）。第 8 天泼尼松反应不良（PPR）与无复发生存率降低相关（25% vs 79.5%，P=.025）。整个组的 4 年无事件生存率（EFS）和总生存率（OS）分别为 60.8%和 64.9%，而 GPR 且 EOI 时缓解的患者（n=15）的 EFS 为 80%，而 PPR 或诱导失败的患者为 16.7%。在儿科 MPAL 中，靶向淋巴细胞的化疗似乎有良好的生存结果。然而，第 8 天的 PPR 或 EOI 骨髓阳性可能是更积极治疗的指征。
