Lymphoproliferative diseases in disorders of the immune system.
Severe immunodeficiency diseases are complicated by the development of malignancies of the immune system itself, mainly non-Hodgkin's lymphoma (NHLs). These occur in immunosuppressed organ allograft recipients; in cancer chemotherapy patients; in patients with primary immunodeficiency diseases; in chronic dialysis patients; in victims of the Acquired Immunodeficiency Syndrome (AIDS); and in persons with various autoimmune diseases either untreated or given immunosuppressive therapy. Most of these NHLs are reticulum cell sarcomas or immunoblastic sarcomas. Immunologically, most are of B-cell origin. Most tumors are extranodal in distribution. They show a remarkable predilection for the brain. Possible causes of the NHLs include oncogenic viruses; disturbed immune surveillance; chronic antigenic stimulation; impaired immunoregulation; carcinogenic effects of immunosuppressive, cytotoxic or other drugs; and genetic susceptibility to lymphomagenesis.
Penn
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Department of Surgery, University of Cincinnati Medical Center, OH 45267-0558.
D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology; D008232:Lymphoproliferative Disorders / Q000209:etiology*