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lüll Hemophagocytic syndrome: a review of 18 pediatric cases Chen CJ; Huang YC; Jaing TH; Hung IJ; Yang CP; Chang LY; Lin TYJ Microbiol Immunol Infect 2004[Jun]; 37 (3): 157-63This retrospective study included 18 pediatric cases (median age, 3 years) with pathologically proved hemophagocytic syndrome (HPS) from a single institution during 1992 and 2001. There were 9 males and 9 females. Prolonged fever, cytopenia, liver dysfunction and hepatomegaly were the most common features at presentation. Sixteen (88.9%) cases were previously healthy. The case fatality rate was 61.1%, and all fatal cases died within 2 months of disease onset. The infectious agents associated with HPS were identified in 11 cases (61.1%), and 8 (72.7%) of them had evidence of Epstein-Barr virus (EBV) infection or reactivation. Underlying immunologic disorder or neoplastic disease was identified in 11.1% of the cases. Children less than 3 years of age with HPS were more vulnerable to neutropenia-associated bloodstream infection (85.7% vs 27.3%; p=0.025). Pseudomonas aeruginosa (3) and Candida tropicalis (2) were the 2 most commonly isolated pathogens. Regarding specific management of HPS, intravenous immunoglobulin and steroids were the first-line agents and were administered in 16 cases and 11 cases, respectively, while etoposide was administered in 5 refractory cases during the late phase of disease. Most HPS occurred in previously healthy children, and a substantial proportion of cases rapidly progressed to death. Most cases were associated with viral infection, particularly EBV, and young children tended to develop neutropenia-associated bacteremia during the active phase of the disease.|*Histiocytosis, Non-Langerhans-Cell/mortality/physiopathology/therapy[MESH]|Adolescent[MESH]|Child[MESH]|Child, Preschool[MESH]|Epstein-Barr Virus Infections/complications[MESH]|Female[MESH]|Herpesvirus 4, Human/isolation & purification[MESH]|Humans[MESH]|Infant[MESH]|Infections/complications/microbiology/virology[MESH]|Male[MESH]|Retrospective Studies[MESH]|Treatment Outcome[MESH] |