Clinical analysis of 18 adult patients with hemophagocytic syndrome / 肿瘤研究与临床

ABSTRACT

Objective To analyze the adult hemophagocytic syndrome' s pathogeny,clinical features,prognostic factors and therapeutic options.Methods 18 cases of adult hemophagocytic syndrome were analyzed,the Kaplan-Meier analysis was used to investigate the total survival rate,and 17 clinical pathological factors and clinical treatment methods which may influence survival were analyzed by Log-rank test in the univariate analysis.Results In this group of patients,EBV infection and malignant lymphoma were the most common initiating diseases.The most common clinical features were peripheral cytopenia in two or three lineages (100 ％),fever (83 ％),splenomegaly (78 ％),swollen lymph nodes (56 ％).The mortality rates as high as 66.7 ％.The median survival time was 7.4 weeks.One-way ANOVA results showed that the initial symptoms as fever (P =0.039),age ＞ 30 years old (P =0.031),enlargement of the liver (P =0.041),Hb ＜ 100 g/L and Ph ＜ 50 g/L (P =0.039) were relevant prognostic factors.Conclusion Adult hemophagocytic syndrome patients with fever as the initial symptoms,age ＞ 30 years old,liver enlargement,Hb ＜ 100 g/L,Plt ＜ 50 g/L indicates poor prognosis,thus these patients having HPS risk factors should be given active chemotherapy and supportive therapy.