Lymphoma complicating immunodeficiency disorders

An increased incidence of lymphoma is seen in various types of immune deficiency or dysregulation, including congenital immune deficiency diseases, organ transplantation with iatrogenic immunosuppression, autoimmune disorders, endemic Burkitt's lymphoma, and AIDS-associated lymphoma.

The lymphomas seen in various immunodeficiency disorders share certain features. Thus, all consist of high-grade, B-cell tumors, with predilection for extranodal involvement, and short survival. However, certain biologic and clinical differences are also apparent. Thus, while the transplantation-associated lymphomas are uniformly associated with the Epstein-Barr Virus (EBV), this association is not seen in all systemic AIDS-related lymphomas, nor in all congenital immune deficiency disorders. Likewise, while c-myc activation has been described in certain types of HIV-related lymphomas, c-myc dysregulation has not been reported in association with organ transplantation.

The study of lymphomatous disease in various states of immune dysregulation may allow an understanding of the diverse pathogenic mechanisms operative in the development of malignant lymphoma in man.