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Hematologic

This is a Blood condition.

Bernard-Soulier Syndrome, BSS

What is BSS?

Dogs affected by Bernard-Soulier Syndrome (BBS) have increased bleeding tendencies. The GP9 gene encodes a subunit of a platelet surface membrane glycoprotein complex; this functions as a receptor for von Willebrand factor (VWF), which initiates the maintenance of blood clotting (hemostasis) after injury.

What are the signs & symptoms that develop in affected dogs?

Signs of this condition include nose bleeds (epistaxis), gingival bleeding, blood in the urine (hematuria), or bleeding after mating. Affected dogs will suffer bleeding tendencies throughout their entire life.

When do signs and symptoms develop?

The typical age at presentation for the first evidence of a bleeding problem is between two to four years of age. However, bleeding may be minimal unless a significant trauma occurs.

How do vets diagnose this condition?

Dogs with BBS show fluctuating changes on complete blood counts (CBCs) which may include enlarged platelets in low numbers, also called macrothrombocytopenia. The most common sign is abnormally large blood clots that result in bruising and bumps beneath the skin (hematoma formation) after they have blood drawn. Genetic testing may reduce the incidence of misdiagnosis as another platelet disorder such as immune-mediated thrombocytopenia (IMTP).

How is this condition treated?

Currently, treatment for BBS focuses on prevention of bleeding episodes, vigilant monitoring, and quick treatment of spontaneous bleeds. Dogs with BBS are at an increased risk for uncontrollable bleeding during surgical procedures. Veterinarians should be informed so that appropriate blood products are on hand in case a transfusion is required for affected dogs. Vets might be cautious in prescribing drugs that have an inhibitory effect on platelet aggregation.

What actions should I take if my dog is affected?

  • Prevention is key! Minimizing the risk of trauma and informing your veterinarian so that surgeries can be carefully planned are the best ways to prevent a catastrophic outcome.
  • BSS may be life-threatening in certain situations. Be aware of the location of the nearest emergency veterinary hospital, and please seek veterinary care immediately.
  • Follow all recommendations from your veterinarian regarding medications that may impact platelet function.