Factor V inhibitor associated with cold agglutinin disease.
An 81-year-old man known to have a stable cold agglutinin syndrome presented with a progressive cerebral hemorrhage. Coagulation tests revealed prolonged APTT and prothrombin time and severely decreased factor V activity, which could not be normalized by mixing with normal plasma. The patient appeared refractory to substitution with fresh-frozen plasma, suggesting the presence of a circulating inhibitor specific for factor V. To our knowledge, this is the first case of a lymphoproliferative disease leading to a cold agglutinin syndrome and a putative inhibitor of factor V. In patients with paraproteinemia presenting with bleeding diathesis, the presence of a circulating inhibitor of a specific coagulation factor must be considered.
van Spronsen
D J
DJ
Department of Internal Medicine, Catharina Hospital, Eindhoven, The Netherlands.
Oosting
J D
JD
Hoffmann
J J
JJ
Breed
W P
WP
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D002543:Cerebral Hemorrhage / Q000150:complications; D005165:Factor V / Q000037:antagonists & inhibitors*; D017809:Fatal Outcome; D006801:Humans; D008297:Male
D005165:Factor V