title: Report of a factor VIII inhibitor in a patient with autoimmune lymphoproliferative syndrome.
abstract: The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lymphoproliferative syndrome (ALPS) who developed an inhibitor to factor VIII. ALPS is a disease characterized by defective lymphocyte apoptosis due to inherited mutations in genes that regulate apoptosis, with the resulting enlargement of lymphoid organs and a variety of autoimmune manifestations. Published 2000 Wiley-Liss, Inc.
authors.affiliation: Hematology Service, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: B S
authors.initials: BS
authors.lastname: Fang
authors.forename: M C
authors.initials: MC
authors.lastname: Sneller
authors.forename: S E
authors.initials: SE
authors.lastname: Straus
authors.forename: L
authors.initials: L
authors.lastname: Frenkel
authors.forename: J K
authors.initials: JK
authors.lastname: Dale
authors.forename: M E
authors.initials: ME
authors.lastname: Rick
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood; D001772:Blood Cell Count; D002648:Child; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D005260:Female; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000276:immunology*
chemical_list: D005169:Factor VIII