Premium
Report of a factor VIII inhibitor in a patient with autoimmune lymphoproliferative syndrome
Author(s) -
Fang Bruno S.,
Sneller Michael C.,
Straus Stephen E.,
Frenkel Lawrence,
Dale Janet K.,
Rick Margaret E.
Publication year - 2000
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/1096-8652(200007)64:3<214::aid-ajh14>3.0.co;2-t
Subject(s) - autoimmune lymphoproliferative syndrome , medicine , autoimmune disease , immunology , lymphoproliferative disorders , disease , lymphoproliferative disease , apoptosis , lymphoma , antibody , biology , programmed cell death , fas receptor , genetics
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. Am. J. Hematol. 64:214–217, 2000. Published 2000 Wiley‐Liss, Inc.