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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 evaluate the efficacy and safety of chemotherapy in children with hemophagocytic lymphohistiocytosis (HLH) with abnormal liver function. Methods According to the guideline of HLH-04 chemotherapy regimen composed of dexamethasone, etoposide and cyclosporine for children with non-tumor related HLH who were treated from March 2004 to April 2008, The therapeutic effects were evaluated and the changes of serum ALT, Alb and Fib were monitored before treatment, 2 weeks after treatment and 8 weeks after treatment. Results A total of 60 cases of HLH patients had abnormal liver function before immunochemotherapy. There were 47 cases of ALT increase, 58 cases of Alb reduction and 38 cases of Fib reduction. 42 cases (70%) related to virus infection, 1 case (1.7%) related to fungal infection and 17 cases (28.3%) with unknown cause. 55 of 60 patients were clinically effective in induction therapy for 4 weeks, 15 patients gave up treatment, and 45 patients completed 8 weeks of induction therapy according to the unified protocol (of which 42 patients had no active disease and 3 patients had activity The changes of ALT, Alb and Fib in the 45 children after 2 weeks and 8 weeks of treatment were all significantly improved. Compared with those before treatment, the differences between the two groups were statistically significant (P0.01). Conclusion HLH-04 chemotherapy is effective in treating HLH, meanwhile it also has good safety in children with abnormal liver function before chemotherapy. Disease at the beginning of liver dysfunction is not a contraindication for immunotherapy, liver function monitoring on the basis of adequate and timely application as possible.