儿童嗜血细胞性淋巴组织细胞增生症预后危险因素分析

发布时间：2018-02-09 01:27

  本文关键词： 嗜血细胞性淋巴组织细胞增生症 儿童 危险因素 预后　出处：《临床血液学杂志》2015年06期 　论文类型：期刊论文

【摘要】：目的:通过分析嗜血细胞性淋巴组织细胞增生症(HLH)患儿的临床表现、诊断标准、治疗以及预后情况,探讨影响HLH预后的危险因素。方法:回顾性分析65例HLH患儿的临床特征、实验室检查结果、治疗方案及预后。结果:65例HLH患儿中,男女之比为1.71∶1.00,发病中位年龄26个月。病因以EB病毒相关性HLH最为常见,共35例(53.85%),风湿免疫性疾病相关HLH 2例(3.08%),原发性HLH 2例(3.08%)。临床表现以发热65例(100%),肝脏肿大63例(96.92%),脾脏肿大54例(83.08%)最为常见。实验室检查以起病时肝功能异常最为明显,铁蛋白升高65例(100%),外周血两系以上细胞减少55例(84.62%),纤维蛋白原降低55例(84.62%),高甘油三酯血症(TG≥3.0mmol/L)47例(72.31%),骨穿发现嗜血现象39例(60.94%)。单因素分析显示,出血倾向、低白蛋白血症及总胆红素升高是影响HLH预后的危险因素;多因素Logistic分析显示,出血倾向及低白蛋白血症是影响HLH预后的独立危险因素。结论:HLH病因复杂,临床表现和实验室检查异常多样,预后凶险,早期诊断和规范治疗十分重要。出血倾向和低白蛋白血症是影响HLH预后的危险因素。
[Abstract]:Objective: to investigate the risk factors influencing the prognosis of HLH by analyzing the clinical manifestations, diagnostic criteria, treatment and prognosis of hemophilic lymphohistiocytosis. Methods: the clinical features of 65 cases of HLH were retrospectively analyzed. Results among 65 children with HLH, the ratio of male to female was 1.71 to 1.00, and the median age was 26 months. Epstein-Barr virus-associated HLH was the most common etiology. A total of 35 cases (53.85%), 2 cases of HLH associated with rheumatic immune diseases (3.08), 2 cases of primary HLH (3.08%), 65 cases of fever (65 cases), 63 cases of hepatomegaly (96.92%), 54 cases of splenomegaly (83.08%) were the most common clinical manifestations. There were 65 cases of elevated ferritin, 55 cases of reduction of peripheral blood cells above two lineages (84.62%), 55 cases of fibrinogen reduction (84.62%), 47 cases of hypertriglyceridemia (TG 鈮，

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