Immunodeficiency-associated lymphomas

Huy Tran, Jamie Nourse, Sara Hall, Michael Green, Lyn Griffiths, Maher K. Gandhi

Research output: Contribution to journalArticle

120 Scopus citations


This article covers lymphoproliferative disorders in patients with primary or acquired immunodeficiencies. Primary immunodeficiences include Ataxia Telangiectasia and X-linked disorders such as Wiskott-Aldrich syndrome. Acquired immunodeficiencies predominantly occur in the setting of infection with the Human Immunodeficiency Virus or arise following immunosuppressive therapy administered after organ transplantation. The rising incidence of HIV throughout the world and the dramatic increase in transplant surgery since the 1990's suggest that these lymphomas will remain an important health problem. Evidence for lymphoma developing as a result of treatment with methotrexate or Tumour Necrosis Factor Antagonists for autoimmune entities will also be reviewed. The lymphoproliferations that occur with immunodeficiency are extremely heterogenous. In part this reflects the diversity of the causal immune defect. The most striking clinical characteristic is the high frequency of extranodal disease. Frequently, these lymphomas are driven by viruses such as Epstein-Barr virus (EBV), although the lack of EBV in a proportion indicates that alternate pathways must also be involved in the pathogenesis. Lastly, discussion will centre on mechanisms utilized by lymphomas in the immunodeficient as these may have applications to lymphomas in the "immunocompetent", by serving as a paradigm for the altered immunoregulatory environment present in many lymphoma sub-types.

Original languageEnglish (US)
Pages (from-to)261-281
Number of pages21
JournalBlood Reviews
Issue number5
Publication statusPublished - Sep 1 2008



  • Ataxia Telangiectasia
  • Combined variable immunodeficiency disorder
  • Diffuse large B cell lymphoma
  • Epstein-barr virus
  • Hodgkin's lymphoma
  • Human herpes virus 8
  • Human immunodeficiency virus
  • Immunodeficiency
  • Infliximab
  • Lymphoma
  • Methotrexate
  • Post-transplantation lymphoproliferative disorder
  • Primary central nervous system lymphoma
  • Primary effusion lymphoma
  • Rituximab

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Tran, H., Nourse, J., Hall, S., Green, M., Griffiths, L., & Gandhi, M. K. (2008). Immunodeficiency-associated lymphomas. Blood Reviews, 22(5), 261-281.