Immunodeficiency and its relation to lymphoid and other malignancies
- Cite this article as:
- Tossing, G. Ann Hematol (1996) 73: 163. doi:10.1007/s002770050221
The reasons why immunodeficiency leads to malignant disorders are multifactorial. The overall incidence of malignancies in persons infected with the human deficiency virus (HIV) is estimated to be 40%. Other infecting agents, especially herpesvirus species, play a pivotal role in HIV-associated non-Hodgkin's lymphoma (NHL) and Kaposi's sarcoma (KS). Mucosaassociated lymphatic tissue (MALT) lymphoma in the stomach may be a result of a chronic gastritis caused by Helicobacter pylori, a gram-negative bacterium. The Epstein-Barr virus (EBV) genome can be found in a high percentage of lymphoma cells of HIV-NHL (nearly 100% in the primary lymphoma of the CNS and about 50% in all other lymphoma entities). In body-cavity based NHL, characterized by the absence of EBV and c-myc oncogen, sequences of a herpesvirus were identified which corresponds to the gamma-herpes viremia found in KS. The Kaposi's sarcoma-associated herpesvirus (KSHV) was usually present in primary-effusion lymphoma (PEL). HIV-infection, which causes multiple dysfunctions within the immune system, triggers the cytokine dysregulation. An abnormal endogenous interferon (IFN)-alpha production is observed in HIV-infected patients with KS, especially in the later natural course of the disease. A monitoring of the IFN-system by MxA, a protein specifically induced by IFN's of type I, may be a necessary stratum of identifying patients who show superior effects and the greatest clinical benefit from treatment with IFN-alpha.