Qin, Li, Yun-Sheng, Chen, Shi-Lin, Liu, Si-Xi, Liu, Xi-Min, Fang, Wei-Guo, Yang, Yue-Jie, Zheng, Wen-Jian, Wang, Ji-Kui, Deng, Jun, Yang, Fei-Qiu, Wen, and Hui-Rong, Mai
To analyze the risk factors affecting prognosis of children with hemophagocytic lymphohistiocytosis (HLH).The clinical manifestations and laboratory data of 143 HLH children who met the HLH-2004 diagnostic criteria in Shenzhen Children's Hospital from January 2009 to May 2017 were retrospectively analyzed, and the independent factors affecting prognosis were also analyzed.The median age of 143 HLH children was 1.9 (0.1-14.3) years old, and the median follow-up time was 6.7 years (1 day - 11.9 years). The overall survival rate of 1 month, 1 year, and 10 years was (87.4±5.5)%, (81.1±6.5)%, and (81.1±6.5)%, respectively. The deaths occurred within 1 year after onset. Multivariate analysis showed that central nervous system (CNS) involvement (P=0.047), low hemoglobin (P=0.002), prolonged activated partial thromboplastin time (APTT) (P0.001), high triglyceride (P=0.005) were all the independent risk factors affecting survival of the children. Receiver operating characteristic curve indicated that APTT (AUC=0.753, P0.001) was more valuable than other risk factors in predicting death of the children. The cut-off value of APTT was 56.6 s, and the sensitivity and specificity of which was 55.6% and 89.7%, respectively.Hypohemoglobinemia, prolonged APTT, hypertriglyceridemia, and CNS involvement the risk factors affecting prognosis of HLH, and prolonged APTT shows a strong predictive value for death.儿童噬血细胞性淋巴组织细胞增生症预后相关因素分析.分析噬血细胞性淋巴组织细胞增生症(HLH)患儿的预后相关危险因素.回顾性分析深圳市儿童医院2009年1月至2017年5月143例符合HLH-2004诊断标准的HLH患儿临床表现及实验室检查,分析影响患儿预后的独立危险因素.患儿的中位年龄为1.9(0.1-14.3)岁,中位随访时间为6.7年(1天-11.9年);1个月、1年和10年总生存率分别为(87.4±5.5)%、(81.1±6.5)%和(81.1±6.5)%,死亡集中发生在发病后1年内;多因素分析结果显示,中枢神经系统受累(P=0.047)、血红蛋白降低(P=0.002)、活化部分凝血活酶时间延长(P0.001)、高甘油三酯(P=0.005)均是影响患儿生存的独立危险因素;受试者工作特征曲线分析提示,活化部分凝血活酶时间(AUC=0.753,P0.001)较其他危险因素预测死亡的价值更大,其预测死亡的截断值为56.6 s,灵敏度和特异度分别为55.6%和89.7.血红蛋白降低、活化部分凝血活酶时间延长、高甘油三酯及中枢神经系统受累是影响HLH预后的危险因素,其中活化部分凝血活酶时间延长对死亡具有较强的预测价值.