ABSTRACT

Impaired immunity resulting from acquired or primary immune deficiency (PID), immunosuppression (IS), or autoimmunity (AI) predisposes to infection and malignancy. Infection and chronic antigenic stimulation are important etiologic factors for malignancies arising in immunodeficiency (ID) and the malignancies are not the same as those seen in the general population. Lymphomas are the most common neoplasms seen in ID. The commonest infective agent in ID causing cancer is the oncogenic Epstein-Barr virus (EBV or human herpes virus 4 [HHV-4]) present in diffuse large B cell lymphoma (DLBCL), Burkitt lymphoma (BL), Hodgkin disease (HD), and lymphomatoid granulomatosis (LyG). Less commonly involved is the Kaposi sarcoma herpes virus (KSHV or HHV-8) in some DLBCL and primary effusion lymphoma (PEL) or Helicobacter pylori predisposing to marginal zone (MZ) or mucosal-associated lymphoid tissue (MALT) lymphomas.