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Br J Haematol. 2013 Sep;162(5):573-86. doi: 10.1111/bjh.12422. Epub 2013 Jun 11.

Primary immunodeficiencies predisposed to Epstein-Barr virus-driven haematological diseases.

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Paediatric Infectious Diseases Research Centre, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran.


Epstein-Barr virus (EBV), a ubiquitous human herpesvirus, maintains lifelong subclinical persistent infections in humans. In the circulation, EBV primarily infects the B cells, and protective immunity is mediated by EBV-specific cytotoxic T cells (CTLs) and natural killer (NK) cells. However, EBV has been linked to several devastating diseases, such as haemophagocytic lymphohistiocytosis (HLH) and lymphoproliferative diseases in the immunocompromised host. Some types of primary immunodeficiencies (PIDs) are characterized by the development of EBV-associated complications as their predominant clinical feature. The study of such genetic diseases presents an ideal opportunity for a better understanding of the biology of the immune responses against EBV. Here, we summarize the range of PIDs that are predisposed to EBV-associated haematological diseases, describing their clinical picture and pathogenetic mechanisms.


Epstein-Barr virus; haemophagocytic lymphohistiocytosis; lymphoma; primary immunodeficiencies; x-linked lymphoproliferative disease

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