成功治疗儿童罕见髓系/自然杀伤细胞祖细胞急性白血病1例

An Unusual Child Case of Myeloid/Natural Killer Cell Precursor Acute Leukemia Treated Successfully with Acute Myeloid Leukemia-Oriented Chemotherapy

本文总结儿童髓系/自然杀伤细胞祖细胞急性白血病(M/NKPAL)的治疗经验以提高对该病的认识。对1例罕见的3岁8个月女童M/NKPAL合并中枢神经系统白血病进行了确诊分析,并对其治疗经过及长期随访结果进行了总结。结果表明,女童M/NKPAL合并中枢神经系统浸润得到了确诊,其免疫表型特征为CD7,CD33,CD34,CD56和HLA-DR共表达,MPO阴性,其他NK细胞和T、B细胞分化抗原阴性,染色体核型有+8和12p-。采用柔红霉素+阿糖胞苷化疗后达完全缓解,随后应用急性髓系白血病的化疗方案巩固强化治疗5个疗程,化疗方案中均含有大剂量阿糖胞苷,期间共行腰穿及鞘内注射治疗10次。停止治疗后中枢神经系统白血病复发,腰穿及鞘内注射治疗9次后行颅脑放疗36 Gy,取得了长期生存。结论:M/NKPAL是一种罕见的白血病,有特异的免疫表型特征,应用含有大剂量阿糖胞苷的急性髓系白血病化疗方案可能取得较好疗效。

This study was aimed to identify the characteristics of childhood myeloid/natural killer cell precursor acute leukemia（M/NKPAL）,and to summarize the therapeutical experiences of this rare hematologic malignancy.A child case of M/NKPAL accompanied by CNS leukemia was enrolled in this study,the therapeutic regiments and the results of long time following up were analysed and evaluated.The results showed that the unusual child case of M/NKPAL with CNS infiltration was diagnosed,showing immunophenotype of CD7＋,CD33＋,CD34＋,CD56＋,HLA-DR＋,MPO-and negative for other NK cell,T and B cell differentiation antigens;the chromosomal abnormalities were trisomy 8 and deletion of chromosome 12p.The child case was treated with daunorubicin and cytarabine,and achieved complete remission.Then,5 coures of acute myeloid leukemia-oriented chemotherapy were given as consolidation chemotherapy,all of the 5 courses contained high dose cytarabine.This child case was given 9 times of lumbar puncture and intrathecal injection,besides these,this case was also given cranial radiotherapy with a dose of 36 Gy.After treated with these methods,the child case achieved long-term complete remission.It is concluded that the M/NKPAL is a rare disease with distinctive immunophenotypic characteristics,acute myeloid leukemia-oriented chemotherapy regimen with high dose of cytarabine may be able to induce long-term remission.