Infections associated with haemophagocytic syndrome

Summary 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 uncon...

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Bibliographic Details
Published in:The Lancet infectious diseases 2007-12, Vol.7 (12), p.814-822
Main Authors: Rouphael, Nadine G, Dr, Talati, Naasha J, MD, Vaughan, Camille, MD, Cunningham, Kelly, MD, Moreira, Roger, MD, Gould, Carolyn, MD
Format: Article
Language:English
Subjects:
Abdomen
Adult
Biological and medical sciences
Diagnosis, Differential
DNA, Viral - analysis
Epstein-Barr Virus Infections - blood
Epstein-Barr Virus Infections - diagnosis
Fatal Outcome
Female
Fingers - abnormalities
Gastroenterology. Liver. Pancreas. Abdomen
Heart Septal Defects
Herpesvirus 4, Human - genetics
Herpesvirus 4, Human - isolation & purification
Human viral diseases
Humans
Infectious Disease
Infectious diseases
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Lymphocytes
Lymphohistiocytosis, Hemophagocytic - blood
Lymphohistiocytosis, Hemophagocytic - diagnosis
Medical sciences
Other diseases. Semiology
Polymerase Chain Reaction
Viral diseases
Viral diseases with cutaneous or mucosal lesions and viral diseases of the eye
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Summary:Summary 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.
ISSN:1473-3099
1474-4457
DOI:10.1016/S1473-3099(07)70290-6