ABSTRACT

General concepts 393 Molecular histogenesis of immunodeficiency-related 394

lymphoma Molecular pathogenesis of immunodeficiency-related 397

lymphoma Genetic background of the host 397 Antigen stimulation 397 Viral infection 398

Molecular pathways 401 Clinico-pathologic entities 405 Human immunodeficiency virus-related lymphoma 405 Post-transplant lymphoproliferative disorders 407 Non-Hodgkin lymphoma related to other 408

immunodeficiency conditions Key points 408 References 408

The World Health Organization (WHO) classification of hematopoietic tumors recognizes four broad settings of immunodeficiency conditions associated with an increased incidence of lymphoma. These are (1) primary immunodeficiency; (2) infection with the human immunodeficiency virus (HIV); (3) iatrogenic immunosuppression in patients who have received solid organ or bone marrow transplantation; and (4) iatrogenic immunosuppression associated with methotrexate treatment for autoimmune diseases.1