Report of a factor VIII inhibitor in a patient with autoimmune lymphoproliferative syndrome.
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.
Fang
B S
BS
Hematology Service, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA.
Sneller
M C
MC
Straus
S E
SE
Frenkel
L
L
Dale
J K
JK
Rick
M E
ME
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*
D005169:Factor VIII