ABSTRACT
Objecfive To investigate the clinical characteristics,treatment and prognosis of autoimmune diseases associated and non-autoimmune diseases associated hemophagocytic syndrome.Methotis Clinical records of 15 cases witll secondary hemophagocytic syndrome'were collected and the relations with treatment and prognosis was analyze.The similarities and differences between autoimmune disease associated bemophagocytic syndrome (group A)and non-autoimmune disease associated hemophagocytic syndrome (group B)were compared.Fisher exact test,t test and Willcoxen test were used for statistical analysis.Results Both groups had fever,bleeding,jaundice,hepatosplenomegaly,and arthralgia,skin rash and positive of autoantibodies in group A were discovered specifically.But in group B,the patients with icterus were mo common(38% vs 100%,p=0.018).There was no significant difference in their laboratory data and prognosis when compared between the two groups(P>0.05).The patients who received corticosteroids and IVIG and/or immunosuppressive agents had better prognosis(P<0.05).Conclusion Except for icterus there is no significant difference in clinical features and laboratory data among autoimmune disease associated hemophagocytic syndrome and other secondary hemophagocytic syndrome.And the therapy with corticosteroids combined with IVIG and/or immunosupprcssive agents is effective.