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

研究成果: 雜誌貢獻回顧型文獻

57 引文 (Scopus)

摘要

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.
原文英語
頁(從 - 到)157-163
頁數7
期刊Journal of Microbiology, Immunology and Infection
37
發行號3
出版狀態已發佈 - 六月 2004
對外發佈Yes

指紋

Hemophagocytic Lymphohistiocytosis
Pediatrics
Neutropenia
Candida tropicalis
Epstein-Barr Virus Infections
Hepatomegaly
Intravenous Immunoglobulins
Etoposide
Virus Diseases
Bacteremia
Human Herpesvirus 4
Pseudomonas aeruginosa
Liver Diseases
Fever
Retrospective Studies
Steroids
Mortality
Infection

ASJC Scopus subject areas

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

引用此文

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.

Hemophagocytic syndrome : A review of 18 pediatric cases. / Chen, Chih Jung; Huang, Yhu Chering; Jaing, Tang Her; Hung, Iou Jih; Yang, Chao Ping; Chang, Luan Yin; Lin, Tzou Yien.

於: Journal of Microbiology, Immunology and Infection, 卷 37, 編號 3, 06.2004, p. 157-163.

研究成果: 雜誌貢獻回顧型文獻

Chen, CJ, Huang, YC, Jaing, TH, Hung, IJ, Yang, CP, Chang, LY & Lin, TY 2004, 'Hemophagocytic syndrome: A review of 18 pediatric cases', Journal of Microbiology, Immunology and Infection, 卷 37, 編號 3, 頁 157-163.
Chen CJ, Huang YC, Jaing TH, Hung IJ, Yang CP, Chang LY 等. Hemophagocytic syndrome: A review of 18 pediatric cases. Journal of Microbiology, Immunology and Infection. 2004 6月;37(3):157-163.
Chen, Chih Jung ; Huang, Yhu Chering ; Jaing, Tang Her ; Hung, Iou Jih ; Yang, Chao Ping ; Chang, Luan Yin ; Lin, Tzou Yien. / Hemophagocytic syndrome : A review of 18 pediatric cases. 於: Journal of Microbiology, Immunology and Infection. 2004 ; 卷 37, 編號 3. 頁 157-163.
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