Lu Wen-Xian, Luo Jian-Ming
Department of Pediatrics, First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2012 Aug;14(8):593-7.
To study the main factors influencing prognosis of hemophagocytic lymphohistiocytosis (HLH) in children by summarizing the clinical features of HLH and investigating the relationship between relevant factors and prognosis.
The medical data of 63 children with HLH were retrospectively reviewed. Kaplan-Meier method was employed to draw survival curves. Factors influencing prognosis were assessed with Cox univariate analysis, and Cox multivariate analysis was done on statistically significant factors.
The 3-year and 5-year survival rates were both 62.9%. The survival rate decreased from 98.4% at 1 day after definite diagnosis to 73.2% at 4 months. Univariate analysis demonstrated only one factor, which was that the condition of platelet recovery after treatment of 2 to 3 weeks was significantly related to prognosis (P=0.002). In children receiving etoposide therapy, temperature recovery after one day of treatment was significantly related to prognosis (P=0.016).
Children with HLH have a satisfactory prognosis, but the survival rate reduces rapidly in the first 4 months after definite diagnosis. Platelet recovery after treatment of 2 to 3 weeks and temperature recovery after one day of treatment are factors influencing prognosis of HLH in children.
通过总结噬血细胞性淋巴组织细胞增生症（HLH）患儿的临床特征，探讨相关因素与预后的关系，研究影响儿童HLH预后的主要因素。
回顾性分析63例HLH患儿的临床资料。采用Kaplan-Meier法绘制生存曲线。用Cox单因素分析评估影响预后的因素，对有统计学意义的因素进行Cox多因素分析。
3年和5年生存率均为62.9%。确诊后1天生存率为98.4%，4个月时降至73.2%。单因素分析显示仅一个因素，即治疗2至3周后血小板恢复情况与预后显著相关（P=0.002）。接受依托泊苷治疗的患儿，治疗1天后体温恢复与预后显著相关（P=0.016）。
HLH患儿预后满意，但确诊后前4个月生存率迅速下降。治疗2至3周后血小板恢复及治疗1天后体温恢复是影响儿童HLH预后的因素。
