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Omenn disease: termination in lymphoma
Pediatric Pathology
|January 1, 1985
Abstract:
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.