Infections associated with haemophagocytic syndrome

• Published in: The Lancet infectious diseases Vol. 7; no. 12; pp. 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: Journal Article Conference Proceeding
• Language: English
• Published: London Elsevier Ltd 01.12.2007; Lancet Publishing Group; Elsevier Limited
• 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; 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; Splenomegaly; Liver; Splenic disease; Hepatic disease; Autoimmune disease; Hemopathy; Epstein Barr virus; Hepatitis; Hemophagocytic lymphohistiocytosis; Hepatomegaly; Rare disease; Gammaherpesvirinae; Immunopathology; Parvovirus; Herpesviridae; Retroviridae; Herpes; Malignant tumor; Histiocytosis; Lentivirus; Fever; Infection; Virus; Hemophagocytic syndrome; Treatment; Parvoviridae; Viral disease; Influenza; Bacteriosis; Digestive diseases; Human immunodeficiency virus; Cancer; Parvovirinae
• ISSN: 1473-3099; 1474-4457
• DOI: 10.1016/S1473-3099(07)70290-6

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.