儿童严重急性高白细胞白血病致死性并发症诊治策略

The management of severe hyperleukocytosis acute leukemia with lethal complications in children

目的探讨儿童严重急性高白细胞白血病(外周血WBC>300×10^9·L^-1)早期致死性并发症的诊治及预后。方法回顾性分析儿科收治的严重急性高白细胞白血病患儿的临床表现、实验室检查、治疗方法及转归。结果 13例患儿确诊时年龄5个月至13岁,初诊时外周血白细胞最高达(301~1 350)×10^9·L^-1,骨髓MICM分型确诊为急性白血病(其中急性淋巴细胞白血病11例,急性髓系白血病2例)。急性淋巴细胞白血病予泼尼松治疗(小剂量开始,逐渐加量),急性髓系白血病予羟基脲治疗,并予水化、别嘌醇降尿酸及对症治疗,7例合并颅内出血的患儿出现不同程度意识障碍,其中3例合并严重颅高压死亡,1例同时合并弥散性血管内凝血及肿瘤溶解综合征死亡;4例合并肿瘤溶解综合征,2例死亡;2例合并呼吸衰竭,1例死亡。结论儿童严重高白细胞白血病早期病死率高,迅速降低循环中白细胞数量,密切监测并防治早期致死性并发症可提高其生存率。

Objective To investigate the diagnosis,treatment and prognosis of severe hyperleukocytosis acute leukaemia(HAL)with lethal complications in children.Methods The clinical data of children with severe HAL who was admitted in the first affiliated hospital of Sun Yat-sen university from January 2013 to June 2018 were retrospectively analyzed.Results Thirteen children were enrolled,aged from 6 months to 13 years,with pre-diagnosed course of 1 weeks to 2 months.The maximum number of white blood cells(WBC)was 301×10^9/L-1350×10^9/L at first diagnosis.The bone marrow MICM classification was diagnosed as acute leukemia(11 cases of acute lymphoblastic leukemia,and 2 cases of acute myeloid leukemia).The cases of acute lymphoblastic leukemia were treated with prednisone(low dose initially and increased gradually),while the others were treated with hydroxyurea.Hydration and allopurinol and other symptomatic treatments were used in all cases.Seven cases compicated with intracranial hemorrhage,3 cases with deep coma and died of severe cranial hypertension,1 case died of disseminated intravascular coagulation(DIC)and tumor lysis syndrome(TLS).Four children complicated with oncolytic syndrome and 2 died.Two children presented respiratory failure and 1 died.Conclusions Severe HAL in children is easily associated with lethal complications and poor prognosis.Rapid reduction of WBC and preventing early lethal complications may improve the survival.