Omenn Disease: Termination in Lymphoma

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 iinfection f i o m vaccination with a live virus to which he did not produce an antibody. H e received transfer factor and irradiated leukoyte 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 pathopesis remains unknown.

Key Words:

• Omenn's disease
• lymphoma
• transjer factor
• histiocytosis
• autosomal-recessive inheritance