Analysis About Prognostic Factors In Children With Hemophagocytic Lymphohistiocytosis

Objective: To summarize the clinical features of hemophagocyticlymphohistiocytosis (HLH) in children, analyze the long-term survival rate, andexplore the prognostic risk factors for the children with hemophagocyticlymphohistosis.Method: We retrospectively reviewed the medical records of childrendiagnosed as HLH in our hospital from January,2005to December,2010, andinvestigated the clinical characteristics. Software package of SPSS16.0wasused to statistic analysis, Kaplan-Meier method was employed to survival curves,related factors were assessed with COX univariate analysis, chose the factorswhich were statistically significant, then analyzed with COX multivariateanalysis. Differences were considered to be statistically significant if the P value<0.05.Result:1. Patients were common from1year old to3years old, the ratio ofmale to female was2:1, and the cardinal clinical manifestations were prolongedfever and hepatosplenomegaly, the common laboratory finding includedincreased lactate dehydrogenase, hyperferritinemia, hemophagocytosis in bonemarrow, cytopenia and abnormal liver function.2. The mean survival time was 3.5years, the over3-year and5-year survival were62.9%, and the survival rateat4months was73.2%.3. In the univariate analysis, the account of the plateletafter treatment had prognostic value, which was statistically significant(P=0.002). Among the patients accepted etoposide therapy, the temperature afterone day of being treated had prognostic value, which was statistically significant(P=0.016).Conclusion:1. The survival rate of HLH children decreased sharply in thefirst4months after the definite diagnosis.2. The patients, whose plateletaccounts still decrease or keep a low level after treatment or had persistent feverafter one day of being treated with etoposide, have poor prognosis and low longterm survival rate.