ISSN 2398-2969      

Hemostatic disorder: primary inherited

icanis

Synonym(s): Bleeding disorders


Introduction

  • Cause: inherited.
  • Signs: usually associated with hemorrhage - may present at young age.
  • Diagnosis: clinical signs of bleeding, hematology, coagulation testing, imaging, genetic testing available for some inherited coagulopathies.
  • Treatment: usually symptomatic often requiring blood product administration.
  • Prognosis: depends on underlying cause.

Pathogenesis

Predisposing factors

General
  • Breed (see above)/family history of bleeding problems.
  • Treatment with medications that potentially reduce coagulation including anticoagulants and non-steroidal anti-inflammatory drugs (NSAIDs).

Pathophysiology

  • Inherited bleeding disorders involve two types of coagulopathy:
  • Defective platelet adhesion:
    • von Willebrand's disease (VWD) Von Willebrand's disease is the most common inherited bleeding disorder in dogs. von Willebrand's factor (vWf) is required for platelet adhesion to areas of endothelial damage and is also involved in platelet-platelet adhesion.
    • Thrombocytopathia and reduced platelet function (rare).
    • Endothelial defects (very rare).
  • Coagulation factor deficiency:
    • Inherited deficiencies in certain coagulation factors result in failure of the coagulation cascade (secondary hemolytic defect) and hemorrhage.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Brooks M, Barnes J, Fremont J (2005) Cosegregation of factor VIII microsatellite marker with mild hemophilia A in Golden Retriever dogs. J Vet Intern Med 19 (2), 205-10 PubMed.
  • Callan M B, Giger U & Catalfamo J L (2005) Effect of desmopressin on von Wilebrand factor multimers in Doberman Pinschers with type I von Willebrand disease. Am J Vet Res 66 (5), 861-7 PubMed.
  • Johnstone I (2002) Bleeding disorders in dogs. 1. Inherited disorders. In Practice 24 (1), 2-10 VetMedResource.
  • de Gopegui R R & Feldman B F (1995) Use of blood and blood components in canine and feline patients with hemostatic disorders. Vet Clin North Am Small Anim Pract 25 (6), 1387-1402 PubMed.
  • Fogh J M (1988) A study of hemophilia A in German Shepherd dogs in Denmark. Vet Clin North Am Small Anim Prat 18 (1), 245-254 PubMed.
  • Fogh J M & Fogh I T (1988) Inherited coagulation disorders. Vet Clin North Am Small Anim Pract 18 (1), 231-244 PubMed.
  • Johnstone I B (1988) Clinical and laboratory diagnosis of bleeding disorders. Vet Clin North Am 18 (1), 21-33 VetMedResource.

Other sources of information

  • Carr A P, Nibblett B M & Panciera D L (2009)von Willebrand's disease and other inherited coagulopathies.In:Kirk's Current Veterinary Therapy XIV, Bonagura J D, Twedt D C (eds). Philadelphia: W B Saunders, pp 277-280.

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