摘要
目的探讨噬血性淋巴组织细胞增生症(HLH)的临床特点。方法回顾性分析2009年4月-2010年5月本院血液科收治的20例HLH患儿临床表现及血常规、肝功能、血液生化、病原学、免疫学检测、血清铁蛋白及骨髓涂片检查等实验室指标,参照HLH-2004治疗方案治疗。结果患儿主要表现为持续发热,肝、脾、淋巴结大,全血细胞减少,肝功能受损,凝血障碍,低纤维蛋白血症,高三酯甘油血症,自然杀伤细胞比例降低。12例中性粒细胞<1.0×109L-1,16例Hb<90 g.L-1,18例血小板<100×109L-1,血细胞二系降低者18例,三系同时降低12例。ALT升高20例,AST升高18例,LDH升高20例,胆碱酯酶升高13例。三酰甘油升高18例,血清铁蛋白>1500μg.L-18例,ESR增快9例;CRP增高11例,自然杀伤细胞比例降低12例,骨髓增生活跃17例,增生低下3例,12例骨髓涂片找到噬血细胞。20例发病与感染相关者中,与EB病毒感染相关性噬血细胞综合征3例,巨细胞病毒感染相关4例,铜绿假单胞菌感染1例。治愈3例,好转15例,疗效不佳自动出院2例。结论 HLH临床表现多样,及时诊治可改善预后。
Objective To explore the clinical features and prognosis factors of hemophagocytic lymphohistiocytosis（HLH） in children.Methods Twenty children with HLH from Apr.2009 to May 2010 were retrospectively analyzed,their clinical features,the results of blood routine,liver function,blood biochemicaltest,pathogens,T-cells and natural killer（NK） cells,serum ferritins and bone marrow smear were detected.The dignosis and treatment were according to HLH-2004 protocol.Results Main clinical features included persistent fever,hepatosplenomegaly,lymphadenopathy,hemorrage and NK cell proportion.Characteristic laboratory findings： 12 children decreased neutrophil（1.0×109 L-1）,16 children decreased hemoglobin（90 g·L-1）,18 children decreased platelet（100×109 L-1）,18 children cytopenia 2 cell lines and 12 children cytopenia 3 cell lines in peripheral blood,20 children had increased serum alamine aminiotrasferase,18 children had increased glutamic oxaloacetic transaminase,20 children had increased lactate dehydrogenase,13 children had increased cholinesterase,and 18 children had increased triacylglycerol.Eight children had increased serum ferritin,9 children had increased erythrocyte sedimentation rate,11 children had increased C-reactive protein and 12 children decreased NK cell.Seventeen children bone marrow were hyperplasia,3 children were hypoplasia and 12 cases hemophagocytosis in bone marrow.Epstein-Barr virus（EBV）-CA-IgM positive were 3 cases and cytomegalovirus（CMV）-IgM positive were 4 cases and pseadomonas aeruginosa was 1 case in 20 children.Treatments and effects： 20 cases received antivirus,high dose of immunoglobin,glucocorticoid and etoposide treatment,3 cases cured,15 cases improved,2 case discharged automaticly because of bad effect.Conclusion The clinical symptoms of HLH are multiple and diagnosis and therapy of HLH in time is important.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2011年第3期191-192,195,共3页
Journal of Applied Clinical Pediatrics
基金
郑州市科技攻关项目(074SGYS33199)
