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Emerg Infect Dis. 2000 Nov-Dec;6(6):601-8.

Hemophagocytic syndromes and infection.

Author information

1
Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA. dfisman@hsph.harvard.edu

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. HLH may be diagnosed in association with malignant, genetic, or autoimmune diseases but is also prominently linked with Epstein-Barr (EBV) virus infection. Hyperproduction of cytokines, including interferon-gamma and tumor necrosis factor-alpha, by EBV- infected T lymphocytes may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic T-cell lymphoma and is treated with cytotoxic chemotherapy, while hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection.

PMID:
11076718
PMCID:
PMC2640913
DOI:
10.3201/eid0606.000608
[Indexed for MEDLINE]
Free PMC Article

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