【摘要】 目的探讨儿童噬血细胞综合征(HPS)的临床特点、诊断、治疗及预后。方法回顾分析2002年1月至2007年6月广东省9所医院诊治的68例儿童HPS的临床特点、治疗及预后。结果68例病例中放弃治疗16例(23.5%),死亡9例(13.2%)。2005年前治疗者共4例,1例完全缓解后失访,余3例死亡。2005年后(釆用HLH-04方案)治疗痊愈20例(20/42,47.6%),完全缓解继续治疗中4例(9.5%),完全缓解后失访6例(14.3%),复发治疗中5例(11.9%)。结论广东地区儿童HPS的发病有逐年增多的趋势;其原发病最常见为EBV感染;采用HLH-04方案治疗后疗效有所提高。更多还原

【Abstract】 Objective To investigate the incidence,clinical characteristics, treatment and prognosis of children with hemophagocytic syndrome(HPS).Methods The clinical data of 68 children with HPS diagnosed in 9 hospitals in Guangdong province from Jan 2002 to July 2007 were retrospectively analyzed.Results(1) General state: ①During the year 2002~2004 16 cases were diagnosed,and 52 cases were diagnosed from the year 2005~2007.②Age distribution: 34 cases were under 2 yrs,20 cases were 3~6 yrs,and 14 cases were 7~14 yrs.③Gender distribution: 40 were males and 28 were females.(2) Present symptoms: all patients had prolonged fever; hepatomegaly and splenomegaly were found in 86.8% and 82.4% cases respectively;(3) Laboratory examination: ① Abnormality of peripheral blood cells: 64.7% cases were with leucocytopenia,60.3% with Hb<90 g/L and 64.7% with thrombocytopenia; ② 22.1% cases had FIB<1.5 g/L.③ Hypertriglyceridemia were found in 44% cases(22/50).④ High serum ferritin were found in 58.7% patients.⑤ 60.0% cases(12/20) presented with decreased NK cells.⑥ 91.9% cases had hematophagocyte in their bone marrow smear:(4) Primary diseases: 26 cases(38.2%)were EBV-HLH,7 cases(10.3%) were found been infected by other microorganism: included 2 Cytomegalo virus,2 Aeruginosus Bacillus,1 Escherichiacoli,1Histoplasma capsulatum and 1 Aspergillus;5 were immunologic diseases related(7.4%);3 were lymphoma related(4.4%);2 were acute lymphoblastic leukemia related: 2 were hematopoietic stem cell transplantation related;2 were Chediak-Higashi syndrome;and 21 patients(30.9%)presented with unknown reason.(5) Treatment and results: In all the 68 cases, 16 cases(23.5%) were discharged from hospital without treatment,and 9 cases were dead(13.2%) during hospitalization.Before the year 05,only 4 patients accepted treatment.In these cases,1 case lost tracing after complete remission(CR),the other three died.After the year 2005,20 cases were recovery(20/42,47.6%) and 4 cases(9.5%) continued treatment after achieved CR,1 case failed to follow up after CR,3 cases were dead.Conclusions In Guangdong,the diagnosis of HPS in children were increasingly in recent years;and EBV-HLH is the leading pathogen;and with the introducing of HLH04 project,the therapeutic effect has increased.更多还原