Thrombasthenia

Thrombasthenia is a blood disorder characterized by impaired aggregation of platelets during wound healing. The defect was originally described by Swiss pediatrician Edward Glanzmann. In humans, Glanzmann thrombasthenia is thought to be caused by lack or depletion of glycoproteins alpha IIb and beta 3. The defect causes prolonged bleeding that results in anemia.

A similar autosomal recessive inherited platelet defect called thromboasthenic thrombopathia has been described in dogs. Affected dogs have normal platelet count and are tested negative for von Willebrand disease, but experience uncontrolled nosebleeding that may be associated with vaccination, hypothyroidism, or estrus. Clinical signs of bleeding in affected dogs are usually recognized before one year of age. The disease has been described in the Great Pyrenees, foxhounds, and Otterhound. Similar platelet defect is seen in the Basset Hound. Recent studies suggest that some severe inherited platelet disorders can be corrected using bone marrow transplantation.



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References

  1. Type I Glanzmann's thrombasthenia in a Great Pyrenees dog. M. K. Boudreaux, K. Kvam, A. R. Dillon, C. Bourne, M. Scott, K. A. Schwartz and M. Toivio-Kinnucan
  2. Two Genetic Defects in IIb Are Associated with Type I Glanzmann's Thrombasthenia in a Great Pyrenees Dog: A 14-base Insertion in Exon 13 and a Splicing Defect of Intron 13.
  3. Correction of a large animal model of type I Glanzmann"s thrombasthenia by nonmyeloablative bone marrow transplantation. Experimental Hematology , Volume 31 , Issue 12 , Pages 1357 - 1362 G . Niemeyer
  4. Genetics for Dog Breeders. Roy Robinson
  5. Black's Veterinary Dictionary. Edward Boden, Geoffrey Philip West
  6. Molecular and genetic basis for thrombasthenic thrombopathia in otterhounds. Boudreaux MK, Catalfamo JL.
  7. A guide to hematology in dogs and cats. A. H. Rebar



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