Factor XI deficiency in Kerry Blue Terriers.

A 9-year-old female Kerry Blue Terrier with postoperative hemorrhage and prolonged activated partial thromboplastin and activated clotting times was determined to have factor XI deficiency. Transfusions of fresh-frozen plasma given on 4 consecutive days transiently returned the values for activated clotting time and plasma factor XI activity to within reference range limits and controlled the hemorrhage. Analysis of data from 10 other factor XI-deficient Kerry Blue Terriers with a tendency for mild posttraumatic or postoperative bleeding was suggestive of an autosomal mode of inheritance, with a mild tendency for posttraumatic or postoperative bleeding in homozygous and heterozygous dogs. Factor XI deficiency is the only contact phase protein defect that causes a bleeding disorder in animals, which can be explained by the fact that thrombin is more efficient than factor XIIa in activating factor XI. Factor XIa plays a key role in sustaining coagulation.