Hemophagocytic syndrome in 32 children

Objective To explore the clinical features,etiological factors,diagnosis and treatments in children with hemophagocytic syndromes (HPS).Methods A retrospective study was carried out to analyze etiological factors,clinical symptoms and sighs,laboratory findings and outcomes of 32 children with HPS in our hospital.Results ( 1 ) Etiological factors:virus infection cases accounted for 75.0％ (24/32),in which Epstein-Barr virus infection cases were predominant,making up to 68.8％ (22/32) ; (2) Main clinical features:included persistent fever,hepatosplenomegaly,lymphadenopathy; ( 3 ) Characteristic laboratory findings:included decrease of three lines or two lines of peripheral blood cells,hyperferrttinemia,abnormal liver function and coagulation function,hypertriglycerdema and decreased fibrinogen,hemophagocytosis in bone marrow,high soluble interleukin-2 receptor level,decreased natural killer cell activity.(4) Treatments and outcomes:twenty-four(24/32) virus infection cases received antivirus and high dose of immunoglobulin treatment.Twenty-two cases were treated with glucocorticoid and cyclophosphamide,resulting in 11 cases improvement,5 cases with complete remission,2 cases giving up and 4 death.The other 10 cases were treated under the guide of HLH-2004 protocol treatmemt with 4 cases improved and 6 cases with complete remission.Conclusion HPS is mainly triggered by infections,especially Epstein-Barr virus infection.Serum ferrifin,interleukin-2 receptor level and natural killer cell activity are biomarkers of the disease,so that they should be monitored during the progress of chemotherapy.HLH-2004 protocol is a good guide for the treatment of this disease. Key words: Hemophagocytic syndrome;  Epstein-Barr virus;  Serum ferritin;  Interleukin-2 receptor;  Natural killer cell ; Children