儿童嗜血细胞综合征43例诊治体会

目的:探讨儿童嗜血细胞综合征(HPS)的临床特点、诊断及治疗。方法:对43例儿童HPS的临床特点、实验室检查及治疗方法进行分析。结果:43例儿童均以发热为首要症状,脾脏肿大有7例(16.28%),肝、脾均肿大为31例(72.09%),肝肿大5例(11.63%),淋巴结肿大40例(93.02%),外周血三系细胞减少共28例(65.12%),两系细胞减少共11例(25.58%),肝功能异常谷丙转氨酶升高38例(88.37%),谷草转氨酶29例(67.44%),总胆红素升高15例(34.88%),总蛋白降低30例(69.77%),甘油三脂升高38例(88.37%),血清铁蛋白升高34例(79.09%),纤维蛋白原降低33例(76.74%),骨髓细胞中见嗜血细胞43例(100.00%)。43例儿童嗜血细胞综合征,放弃16例(37.21%),接受治疗27例(62.79%),接受治疗患儿中死亡8例(18.61%),痊愈16例(37.21%),3例正在治疗中(6.98%)。结论:HPS由多种病因所致,发病年龄以6岁前儿童为多。原发病EBV感染占首位,预后凶险,而且病情易复发,年龄越小预后越差,确诊后果断采用HLH-04方案治疗有助于HPS生存率提高。

Clinical analysis of 43 childhood hemophagocytic lymphohistiocytosis

Objective:To investigate the clinical features,diagnosis and therapy of childhood hemophagocytic lymphohistiocytosis.Methods: A study was carried out in clinical characteristics,laboratory examination and treatment of 43 children who hospitalized for hemophagocytic lymphohistiocytosis.Results: All of these 43 cases childhood hemophagocytic lymphohistiocytosis toke fever as the chiefly symptom.Among these cases,7(16.28%) were splenomegaly,hepatomegaly were 5(11.63%),31 of total 43 cases(72.09%) were hepatosplenomegaly and 40 cases(93.02%) had enlargement of the lymphnodes.Cytopenias of 3 lineages in the peripheral blood were 28 cases(65.12%) and 11 cases(25.58%) had affect on 2 lineages,38 cases(88.37%) hypertriglyceridemia,33 cases(76.74%) hypofibrinogenemia,34 cases(79.09%) were elevated levels of ferritin,and all cases(100%) had hemophagocytosis in bone marrow.In addition,liver dysfunction was the common symptoms,the value of GPT elevates in 38 cases(88.37%),29 cases(67.44%) of GOT and total bilirubin elevates in 15 cases(34.88%),total protein degraded in 30 cases(69.77%).In the 43 cases childhood hemophagocytic lymphohistiocytosis,16 cases(37.21%) abandon treatment,treatment 27 cases(62.79%),8 cases(18.61%) died in therapeutic process,16 cases(37.21%) achieve recovery and 3 cases(6.98%)were being treated.Conclusion:Age of onset more than 6 years old(34cases,79.07%),fever,hepatosplenomegaly,lymphadenopathy and cytopenias of 3 or 2 lineages in the peripheralblood are features.EBV infection,28 cases(65.12%),is the most common primary disease,and easy to recurrence of disease difficult to control,the younger the worse the prognosis.Early diagnosis and timely adoption of HLH-04 schema is helpful on improve survival rates of hemophagocytic lymphohistiocytosis.