Journal Article

The Role of Infections in Primary Hemophagocytic Lymphohistiocytosis: A Case Series and Review of the Literature

L. Sung, S. S. Weitzman, M. Petric and S. M. King

in Clinical Infectious Diseases

Published on behalf of Infectious Diseases Society of America

Volume 33, issue 10, pages 1644-1648
Published in print November 2001 | ISSN: 1058-4838
Published online November 2001 | e-ISSN: 1537-6591 | DOI: https://dx.doi.org/10.1086/323675
The Role of Infections in Primary Hemophagocytic Lymphohistiocytosis: A Case Series and Review of the Literature

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There is a paucity of literature addressing infection-related morbidity and mortality in children with primary hemophagocytic lymphohistiocytosis (HLH), a rare condition characterized by abnormal proliferation of macrophages, hypercytokinemia, and T cell immunosuppression. Therefore, a retrospective chart review was done of patients diagnosed with primary HLH over a 15-year period. Significant infections present at diagnosis, during the course of illness, and just prior to death or at autopsy were noted. Of the 18 children identified with primary HLH, an infectious agent was documented at the initial presentation of HLH in 5. Significant infections occurred during therapy in 10 (56%) of 18. Of the 12 fatal cases, invasive infection was the cause of death in 8 children, and 6 of these deaths were directly attributable to invasive fungal infection. Significant infections were common during therapy in children with primary HLH, and fungal infections were an important cause of mortality in this group.

Journal Article.  2505 words.  Illustrated.

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

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