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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。
Objective: To analyze the clinical manifestations, diagnostic criteria, treatment and prognosis of children with hemophilia lymphohistiocytosis (HLH) and explore the risk factors influencing the prognosis of HLH. Methods: A retrospective analysis of 65 cases of HLH children with clinical features, laboratory test results, treatment and prognosis. Results: In 65 children with HLH, the ratio of men to women was 1.71: 1.00, and the median age was 26 months. The most common cause of the disease was EB virus-related HLH, 35 (53.85%), rheumatoid immune disease-related HLH (3.08%), and primary HLH (3.08%). The clinical manifestations were 65 cases (100%) with fever, 63 cases (96.92%) with enlarged liver and 54 cases (83.08%) with enlarged spleen. The liver function abnormalities were most obvious in laboratory examination. Ferritin increased in 65 cases (100%), 55 cases (84.62%) in peripheral blood more than two lines, and 55 cases (84.62%) in fibrinogen decreased There were 47 cases (72.31%) of triglyceridemia (TG≥3.0mmol / L), and 39 cases (60.94%) of smear-induced bloodlust. Univariate analysis showed that bleeding tendency, hypoalbuminemia and total bilirubin were risk factors influencing prognosis of HLH. Multivariate logistic analysis showed that bleeding tendency and hypoalbuminemia were independent risk factors for prognosis of HLH . Conclusions: The etiology of HLH is complex, the clinical manifestations and laboratory tests are abnormally diverse, and the prognosis is dangerous. Early diagnosis and standard treatment are very important. Bleeding tendency and hypoalbuminemia are risk factors for the prognosis of HLH.