Immunodeficiency-Associated Lymphoproliferative Disorders Other Than PTLD (in Primary Immune Deficiency, HIV, and Iatrogenic Conditions)

Abstract

This chapter discusses the three categories of lymphoproliferative disorders arising from immune deficiencies: primary immune deficiency, human immunodeficiency virus (HIV) infection, and iatrogenic medication. Posttransplant lymphoproliferative disorder is discussed in a separate chapter. In all three categories, various forms of immune suppression result in increased risks of developing lymphoproliferative disorders. In primary immune deficiency, compromised humoral immunity, cellular immunity, or both are associated with inherited or congenital disorders that result in increased incidence of lymphoid neoplasms. These lymphomas most commonly arise in children and young adults. In HIV infection, cellular immunities are compromised by direct viral infection of helper/inducer T cells. In conditions associated with iatrogenic medication, lymphomas arise after prolonged immune suppression by cytotoxic chemotherapy or immune modulatory therapy. These drugs suppress and modulate cellular and humoral immunities resulting in immunologic dysregulation and lymphoid hyperactivation. Although the etiologies of the immune deficiencies vary in these disorders, they result in similar consequences with dysregulation and suppression of immune system and increased risk of lymphomas most commonly EBV-driven large cell and aggressive lymphomas.