Abstract

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.

摘要

关于小儿混合表型急性白血病（MPAL）的数据有限，目前尚无全球共识。在这项回顾性研究中，我们分析了在我们研究所诊断为MPAL的儿童的结局。这项研究包括从2009年1月1日至2015年12月31日在印度第三级癌症中心就诊的MPAL≤14岁的儿童。在7年的时间里，有1390例白血病患者向我们研究所提出，其中22例（1.5％）有MPAL。16例（72.7％）的患者患有B /髓样白血病，而4例（18.1％）和2（9％）的患者分别患有T /髓样和B / T白血病。21例患者接受了改良的BFM ALL 95方案治疗。76.1％（n = 16）的患者在第8天的泼尼松龙反应（GPR）良好，并且诱导终止（EOI）骨髓的缓解率达到90.5％（n = 19）。第8天的泼尼松龙不良反应（PPR）与无复发生存期较差有关（25％vs 79.5％，P = .025）。整个组的4年无事件生存率（EFS）和总体生存率（OS）分别为60.8％和64.9％，而GPR和EOI缓解（n = 15）的患者的EFS为80％ PPR或诱导失败的患者为16.7％。淋巴定向化疗在小儿MPAL中被认为具有良好的生存结局。但是，第8天的PPR或EOI骨髓阳性可能是更积极治疗的指征。GPR和EOI缓解（n = 15）的患者的EFS分别为9％和80％，而PPR或诱导失败的患者的EFS为80.8％。淋巴定向化疗在小儿MPAL中被认为具有良好的生存结局。但是，第8天的PPR或EOI骨髓阳性可能是更积极治疗的指征。GPR和EOI缓解（n = 15）的患者的EFS分别为9％和80％，而PPR或诱导失败的患者的EFS为80.8％。淋巴定向化疗在小儿MPAL中被认为具有良好的生存结局。但是，第8天的PPR或EOI骨髓阳性可能是更积极治疗的指征。