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Factor XI deficiency in Kerry Blue Terriers

C Knowler¹, U Giger, W J Dodds

¹Department of Clinical Studies, School of Veterinary Medicine, University of Pennsylvania, Philadelphia 19104-6010.

Journal of the American Veterinary Medical Association

|December 1, 1994

Summary

Factor XI deficiency in Kerry Blue Terriers causes bleeding disorders. Fresh-frozen plasma transfusions can temporarily correct clotting times and control hemorrhage in affected dogs.

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Area of Science:

Veterinary Hematology
Canine Coagulation Disorders

Background:

Factor XI deficiency is a rare inherited bleeding disorder.
It affects the contact phase of coagulation, crucial for sustained clotting.
This condition is characterized by prolonged clotting times and a tendency for hemorrhage.

Observation:

A 9-year-old Kerry Blue Terrier presented with postoperative hemorrhage and prolonged activated clotting times.
The dog was diagnosed with factor XI deficiency.
Treatment involved daily fresh-frozen plasma transfusions over four days.

Findings:

Transfusions normalized activated clotting time and plasma factor XI activity, controlling hemorrhage.
Data from 10 additional affected dogs suggested an autosomal inheritance pattern.

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Both homozygous and heterozygous dogs exhibited a mild bleeding tendency, particularly post-trauma or post-surgery.

Implications:

Factor XI deficiency is the sole contact phase protein defect causing bleeding in animals.
Thrombin's higher efficiency in activating factor XI compared to factor XIIa explains this.
Understanding factor XI's role in coagulation is vital for managing bleeding disorders in dogs.