先天性白血病的临床特点与疗效分析

Clinical characteristic and curative effect analysis of congenital leukemia

目的探讨先天性白血病的诊疗方法及影响其预后的主要因素。方法回顾性收集整理郑州儿童医院2007年7月至2017年12月收治的先天性白血病患儿的临床资料,分析其临床特点、化疗方案及影响其长期生存的因素。结果共收治先天性白血病患儿23例,多以"白细胞增高、皮肤结节、皮肤出血"就诊。其中放弃治疗10例,经随访无一例存活。治疗13例,男7例,女6例,其中有1例为双胎之一。急性髓细胞白血病8例,急性淋巴细胞白血病5例;融合基因检测,1例未做,2例MLL基因阳性,10例MLL基因阴性,未检测出其他融合基因;染色体核型分析1例为21-三体,12例染色体核型正常。13例患儿均接受了对症和/或减量化疗。中位随访时间4年11个月,其中痊愈6例,死亡7例。1例为出生6 d时外院确诊急性淋巴细胞白血病未予及时治疗,于32 d时全身多发包块就诊,检查后确诊并发中枢白血病及睾丸白血病,予较强化疗后合并肺部、脑内重症感染,经美国卢里芝加哥儿童医院远程会诊因感染较重未能挽回生命,余6例患儿均在治疗过程中出现重症感染并多脏器损伤死亡,无一例死于白血病复发。结论减量化疗可能是先天性白血病患儿获益的重要方法。

Objective To investigate the diagnosis and treatment of congenital leukemia and the main factors affecting its prognosis.Methods Clinical data of children with congenital leukemia admitted to Zhengzhou Children′s Hospital from July 2007 to December 2017 were retrospectively collected.Their clinical characteristics,chemotherapy and factors affecting their long-term survival were analyzed.Results A total of 23 cases of congenital leukemia were collected,who mostly complained of"increased white blood cells,skin nodules and skin hemorrhage".Among them,10 cases abandoned the treatment,who all died according to the follow-up results.Among the 13 treated cases,7 cases were male,and 6 were female.One of the 13 cases was a twin.Eight cases were acute myeloid leukemia,and 5 cases were acute lymphoblastic leukemia.One case did not perform the fusion gene test.MLL gene was positive in 2 cases and negative in 10 cases.No other fusion genes were detected.Karyotype analysis showed that 1 case was 21-trisomy and 12 cases had a normal karyotype.All 13 patients received symptomatic and/or reduced chemotherapy.The median follow-up time was 4 years and 11 months.Six cases recovered and 7 cases died during the follow-up.One case was diagnosed with acute lymphoblastic leukemia on the 6th day after the birth in another hospital.He was not treated timely and came to Zhengzhou Children′s Hospital with general contract blocks at the age of 32 days.After checking,he was diagnosed with center leukemia and testicular leukemia.He developed a severe infection in the lung and brain after strong chemotherapy,and died even after the remote consultation with Lurie Children′s Hospital of Chicago.The other 6 cases died of severe infection and multiple organ injuries after chemotherapy,and no one died of leukemia relapse.Conclusion Reduction chemotherapy may be an important treatment to children with congenital leukemia.