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Volume 37, Number 3, June 2004

Hemophagocytic syndrome: a review of 18 pediatric cases

Chih-Jung Chen, Yhu-Chering Huang, Tang-Her Jaing, Iou-Jih Hung, Chao-Ping Yang, Luan-Yin Chang, Tzou-Yien Lin
Divisions of Pediatric Infectious Diseases and Pediatric Hematology-Oncology, Department of Pediatrics, Chang Gung Children¡¦s Hospital, Taoyuan, Taiwan, ROC

Received: June 11, 2003    Revised: July 9, 2003    Accepted: September 25, 2003   


Corresponding author:

Dr. Yhu-Chering Huang, Division of Pediatric Infectious Disease, Department of Pediatrics, Chang Gung Children¡¦s Hospital, 5, Fu-Shin Street, Kweishan, Taoyuan, Taiwan 333, ROC. E-mail: This e-mail address is being protected from spam bots, you need JavaScript enabled to view it




This 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.



Key words:

Child, Epstein-Barr virus, hemophagocytic syndrome



J Microbiol Immunol Infect 2004;37:157-163.