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

Authors

  • Nima Parvaneh,

    Corresponding author
    1. Division of Allergy and Clinical Immunology, Department of Paediatrics, Tehran University of Medical Sciences, Tehran, Iran
    • Paediatric Infectious Diseases Research Centre, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran
    Search for more papers by this author
  • Alexandra H. Filipovich,

    1. Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA
    Search for more papers by this author
  • Arndt Borkhardt

    1. Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, Heinrich-Heine University, Dusseldorf, Germany
    Search for more papers by this author

Correspondence: Nima Parvaneh, Paediatric Infectious Diseases Research Centre, Children's Medical Centre, 62 Gharib St, 14194 Tehran, Iran.

E-mail: nparvaneh@tums.ac.ir

Summary

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.

Ancillary