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

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Abstract

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.

Original languageEnglish
Pages (from-to)157-163
Number of pages7
JournalJournal of Microbiology, Immunology and Infection
Volume37
Issue number3
Publication statusPublished - Jun 2004
Externally publishedYes

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Keywords

  • Child
  • Epstein-Barr virus
  • Hemophagocytic syndrome

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology and Microbiology(all)
  • Microbiology (medical)
  • Infectious Diseases

Cite this

Chen, C. J., Huang, Y. C., Jaing, T. H., Hung, I. J., Yang, C. P., Chang, L. Y., & Lin, T. Y. (2004). Hemophagocytic syndrome: A review of 18 pediatric cases. Journal of Microbiology, Immunology and Infection, 37(3), 157-163.