儿童噬血细胞性淋巴组织细胞增生症的预后因素分析

Prognostic factors for hemophagocytic lymphohistiocytosis in children

摘要:

目的：通过总结儿童噬血细胞性淋巴组织细胞增生症(HLH)的临床特征，分析相关因素与预后的关系，探索影响HLH患儿预后的因素。方法：回顾性分析63例HLH患儿的病例资料，使用Kaplan-Meier法绘制生存曲线，采用COX模型对影响预后的相关因素进行单因素分析，筛选出有意义的因素进行多因素COX回归分析。结果：患儿的3年及5年生存率均为62.9%；确诊后第1天至4个月，生存率由98.4%降至73.2%。影响疾病预后单因素分析中，具有统计学意义的因素只有治疗后2~3周血小板恢复情况（P=0.002）；在使用依托泊苷治疗的患儿中，治疗1 d后体温恢复情况对预后的影响具有统计学意义（P=0.016）。结论：HLH患儿的预后较为满意，但确诊后的前4个月生存率下降快。治疗2~3周后血小板恢复情况以及使用依托泊苷1 d后体温恢复情况影响患儿预后。

Abstract:

OBJECTIVE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.