Infections associated with haemophagocytic syndrome

• Type of Material: Article
• Authors: Nadine Rouphael, Emory University; Naasha Talati, Emory University; Camille Vaughan, Emory University; Kelly Cunningham, Emory University; Roger Moreira, Emory University; Carolyn Gould, Emory University
• Language: English
• Date: 2007-12-01
• Publisher: ELSEVIER SCI LTD
• Publication Version: Final Published Version
• Copyright Statement: © 2007 Elsevier Ltd. All rights reserved.
• Final Published Version (URL): http://dx.doi.org/10.1016/S1473-3099(07)70290-6
• Title of Journal or Parent Work: LANCET INFECTIOUS DISEASES
• Volume: 7
• Issue: 12
• Start Page: 814
• End Page: 822
• Keywords: ACTIVE ANTIRETROVIRAL THERAPY; PATIENT; Life Sciences & Biomedicine; LYMPHOHISTIOCYTOSIS; H5N1 VIRUS; CLINICAL-FEATURES; Infectious Diseases; PARVOVIRUS B19 INFECTION; HIV-INFECTION; HUMAN-IMMUNODEFICIENCY-VIRUS; SPONTANEOUS RESOLUTION; INFLUENZA-A; Science & Technology
• Research Categories: Biology, Virology; Health Sciences, Immunology

Abstract

Haemophagocytic syndrome or haemophagocytic lymphohistiocytosis is a rare disease that is often fatal despite treatment. Haemophagocytic syndrome is caused by a dysregulation in natural killer T-cell function, resulting in activation and proliferation of lymphocytes or histiocytes with uncontrolled haemophagocytosis and cytokine overproduction. The syndrome is characterised by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hyperferritinaemia. Haemophagocytic syndrome can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. Infections associated with haemophagocytic syndrome are most frequently caused by viruses, particularly Epstein-Barr virus (EBV). We present a case of EBV-associated haemophagocytic syndrome in a young adult with no known immunosuppression. We briefly review haemophagocytic syndrome and then discuss its associated infections, particularly EBV and other herpes viruses, HIV, influenza, parvovirus, and hepatitis viruses, as well as bacterial, fungal, and parasitic organisms.