Viral infections associated with haemophagocytic syndrome

Authors

  • Nadine Rouphael Maakaroun,

    Corresponding author
    1. Division of Infectious Diseases, Emory University School of Medicine, Atlanta, GA 30303, USA
    • Division of Infectious Diseases, Emory University School of Medicine, 80 Butler Street, Atlanta, GA 30303, USA.
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    • Assistant Professor.

  • Abeer Moanna,

    1. Division of Infectious Diseases, Emory University School of Medicine, Veterans Affairs Medical Center, Decatur, GA 30033, USA
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    • Assistant Professor.

  • Jesse T Jacob,

    1. Division of Infectious Diseases, Emory University School of Medicine, Atlanta, GA 30303, USA
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    • Assistant Professor.

  • Helmut Albrecht

    1. Division of Infectious Diseases, University of South Carolina, Columbia, SC 29203 USA
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    • Professor of Medicine, Division Chief.


Abstract

Haemophagocytic syndrome (HPS) or haemophagocytic lymphohistiocytosis (HLH) is a rare disease caused by a dysfunction of cytotoxic T cells and NK cells. This T cell/NK cell dysregulation causes an aberrant cytokine release, resulting in proliferation/activation of histiocytes with subsequent haemophagocytosis. Histiocytic infiltration of the reticuloendothelial system results in hepatomegaly, splenomegaly, lymphadenopathy and pancytopenia ultimately leading to multiple organ dysfunctions. Common clinical features include high fevers despite broad spectrum antimicrobials, maculopapular rash, neurological symptoms, coagulopathy and abnormal liver function tests. Haemophagocytic syndrome can be either primary, i.e. due to an underlying genetic defect or secondary, associated with malignancies, autoimmune diseases (also called macrophage activation syndrome) or infections. Infectious triggers are most commonly due to viral infections mainly of the herpes group, with EBV being the most common cause. HPS can be fatal if untreated. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. HPS triggered by viral infections generally does not respond to specific antiviral therapy but may be treated with immunosuppressive/immunomodulatory agents and, in refractory cases, with bone marrow transplantation. Copyright © 2010 John Wiley & Sons, Ltd.

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