化疗对肝功能异常的噬血细胞性淋巴组织细胞增生症的有效性及安全性观察  

Observation of Efficacy and Safety of Chemotherapy on Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in Children

作　　者：贾晨光[1] 杨双[1] 张莉[1] 马洪浩[1] 

机构地区：[1]首都医科大学附属北京儿童医院血液病中心,北京100045

出　　处：《实用儿科临床杂志》2010年第3期170-171,174,共3页Journal of Applied Clinical Pediatrics

摘　　要：目的初步评价化疗对并肝功能异常的噬血细胞性淋巴组织细胞增生症(HLH)患儿的有效性及安全性。方法选取对2004年3月-2008年4月收治的非肿瘤相关HLH患儿,参照由地塞米松、依托泊苷、环孢素组成的HLH-04化疗方案指南,在化疗后第8周对疗效进行评估,并在治疗前、治疗后2周、治疗后8周末监测血清ALT、血清清蛋白(Alb)、血浆纤维蛋白原(Fib)等指标变化。结果共60例HLH患儿在接受免疫化疗前并肝功能异常,其中ALT增高47例,Alb降低58例,Fib降低38例。病毒感染相关42例(70%),真菌感染相关1例(1.7%),不明原因17例(28.3%)。60例患儿中55例在诱导治疗4周中临床有效,15例患儿放弃治疗,45例患儿按统一方案完成了8周诱导治疗(其中42例无活动性病变,3例存在活动性病变),该45例患儿治疗后2周、8周,ALT、Alb、Fib监测指标均明显改善,与治疗前比较,组间差异均有统计学意义(Pa<0.01)。结论HLH-04化疗方案治疗HLH临床疗效显著,同时对化疗前肝功能异常患儿同样具有较好的安全性。病初肝功能异常并非免疫化疗的禁忌证,在监测肝功能的基础上应尽可能按时足量应用。Objective To preliminarily evaluate the efficacy and safety of chemotherapy on hemophagocytic lymphohistiocytosis（HLH） with hepatic dysfunction in children.Methods The children diagnosed as non-malignancy-associated HLH from Mar.2004 to Apr.2008 were selected,and the therapeutic effect was evaluated according to the HLH-04 protocol at the 8th week of chemotherapy,and the level of serum alanine aminotransferase（ALT）,serum albumin（Alb） and plasma fibrinogen（Fib） were detected at pretherapy,2 weeks and 8 weeks of post-treatment.Results Altogether 60 HLH children complicated with hepatic dysfunction before chemotherapy,47 children had increased ALT,58 children had decreased Alb,and 38 children had decreased Fib.Forty-two cases（70%） were virus-associated HLH,1 case（1.7%） was fungi-associated HLH,and 17 cases（28.3%） had unknown origin.Among the 60 children,55 cases showed improvement in the 4 weeks of inductive treatment,15 cases gave up therapy,45 cases completed the 8 weeks of inductive treatment according to the protocol（among these children,42 cases had no active disease,3 cases had active disease）,and these 45 children had obviously improved ALT,Alb and Fib at 2 weeks and 8 weeks of post-treatment,compared with pretherapy,the differences had statistical significance（Pa〈0.01）.Conclusions The chemotherapy of HLH-04,including dexamethasone,etoposide and ciclosporin A,has significant therapeutic effect,meanwhile,it is safe to HLH children with hepatic dysfunction.And hepatic dysfunction is not contraindication of chemotherapy,the therapy should be quantity sufficient and on time,based on monitering the hepatic function.

关 键 词：噬血细胞性淋巴组织细胞增生症 化疗 肝功能 

分 类 号：R725.5[医药卫生—儿科]