Journal Article

Hemophagocytic Syndrome in Children: An Important Diagnostic Consideration in Fever of Unknown Origin

Debra L. Palazzi, Kenneth L. McClain and Sheldon L. Kaplan

in Clinical Infectious Diseases

Published on behalf of Infectious Diseases Society of America

Volume 36, issue 3, pages 306-312
Published in print February 2003 | ISSN: 1058-4838
Published online February 2003 | e-ISSN: 1537-6591 | DOI: http://dx.doi.org/10.1086/345903
Hemophagocytic Syndrome in Children: An Important Diagnostic Consideration in Fever of Unknown Origin

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To study the evolution of hemophagocytic syndrome (HPS) in children, we performed a retrospective review of 19 patients (median age, 17.4 months) in whom an infectious diseases consultation was requested at Texas Children's Hospital during the period of September 1991 through September 2001. Clinical findings consistent with HPS most frequently presented during days 6–14 of illness, concomitant with laboratory abnormalities. Fever was present for a median of 19 days before the diagnosis of HPS. Elevated serum lactate dehydrogenase and ferritin levels were noted in all patients. An infectious agent was identified in 42% of patients; 16% were found to have immunologic or vasculitic disease. HPS is a rare but often fatal disease that can initially present as fever of unknown origin with varying clinical findings, and it can be recognized by physicians who are familiar with the evolution of HPS. It is likely that many of these cases remain undiagnosed because of the HPS's rapidly fatal course.

Journal Article.  3325 words.  Illustrated.

Subjects: Infectious Diseases ; Immunology ; Public Health and Epidemiology ; Microbiology

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