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.%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.
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