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 ra re event with a potentially lethal outcome. Despite its infrequent occurren ce, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently describe d autoimmune lymphoproliferative syndrome (ALPS) who developed an inhibitor to factor VIII, ALPS is a disease characterized by defective lymphocyte ap optosis due to inherited mutations in genes that regulate apoptosis, with t he resulting enlargement of lymphoid organs and a variety of autoimmune man ifestations. Published 2000 Wiley-Liss, Inc.(1)