Acquired immunodeficiency syndrome-related lymphoma [see comments]

Abstract

T immune dysregulation induced by the human immunodeficiency virus (HIV) is fully consistent with the known occurrence of lymphoma in other settings of immunologic compromise. Thus, the incidence of malignancy in congenital immune deficiency diseases is approximately 100 times greater than expected, with malignant lymphoma comprising the majority of these cancers in children with ataxia telangiectasia or Wiskott-Aldrich syndrome,lJ in which the latter are uniformly associated with presence of the Epstein-Barr virus (EBV) genome within tumor cell DNA. Similarly, boys with the X-linked lymphoproliferative syndrome may develop high-grade, B-cell lymphomas after exposure to EBV.3 Individuals with various autoimmune diseases are also at increased risk for developing B-cell lymphoma, as documented in patients with Sjogren's syndrome, Hashimoto's thyroiditis, and Further, in the setting of organ transplantation with iatrogenic immunosuppression and chronic antigenic stimulation, polyclonal lymphoproliferative disorders have been described. True malignant lymphoma may also occur with increased frequency in this ~etting.8.~ Similar to the congenital or autoimmune settings, the transplant-associated lymphomas are usually highgrade, B-immunoblastic lymphomas, with presence of EBV genome within tumor cell DNA. Genotypic monoclonality has been demonstrated in these cases using Southern blot analysis to confirm Ig-gene rearrangement.10 Of interest, increasing degrees of immunosuppression, with regimens containing cyclosporine and/or monoclonal antibodies (Mo-Abs) to induce T-cell depletion have resulted in an increased risk of developing these lymphomas, which occur earlier than usually expected after transplantation.Il With these data in perspective, then, it is not surprising that patients with HIV-induced immune dysregulation might also be at increased risk for development of high-grade, B-cell lymphoma.