Omenn disease: termination in lymphoma.
Two brothers with Omenn's disease were seen at the University of Wisconsin. The first died at 3 months. The second developed a chronic measles infection from vaccination with a live virus to which he did not produce an antibody. He received transfer factor and irradiated leukocyte infusions without benefit. After death a lymphoma was found in virtually all tissues. It is unclear whether the treatment predisposed the child to or caused the cancer. Omenn's disease is an autosomal-recessive disorder of the immune system that leads to early death. The pathogenesis remains unknown.
D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000235:genetics / Q000473:pathology*; D007223:Infant; D008223:Lymphoma / Q000209:etiology* / Q000473:pathology; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology