Hemophagocytic syndrome in 32 children / 中国小儿急救医学

ABSTRACT

ObjectiveTo explore the clinical features,etiological factors,diagnosis and treatments in children with hemophagocytic syndromes (HPS).MethodsA 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 factorsvirus 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 featuresincluded 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.