Clinical characterization of canine platelet procoagulant deficiency (Scott syndrome).

BACKGROUND

Platelet function defects are rare causes of bleeding diatheses; however, disease prevalence might be underestimated because diagnosis requires assessment of specific parameters of platelet activation.

OBJECTIVES

The goal of this study was to characterize the clinical presentation of canine Scott syndrome (CSS), an intrinsic platelet function defect first identified in a closed colony of German Shepherds (GSD).

ANIMALS: Eleven (n = 6 female) client-owned GSD affected with CSS that sought veterinary care for one or more episodes of abnormal bleeding.

METHODS

Retrospective review of all cases of CSS diagnosed through the Comparative Coagulation Laboratory at Cornell University between 2005 and 2011. The diagnosis of CSS was based on 2 measures of platelet procoagulant activity: serum prothrombin consumption and flow cytometric detection of platelet phosphatidylserine externalization after in vitro activation.

RESULTS

Postoperative hemorrhage was the most common sign of CSS, whereas petechiae were not found in any dog. Although most GSD responded to platelet transfusion, refractory epistaxis in 2 GSD was managed by nasal arterial embolization. The CSS trait was not restricted to a single pedigree of related GSD or to a single geographic region.

CONCLUSIONS AND CLINICAL

IMPORTANCE

Unlike thrombocytopenia and platelet aggregation defects, petechiae and other capillary hemorrhage are not typical features of CSS. After preliminary screening to rule out more common causes of hemorrhage, CSS should be considered in the differential diagnosis of recurrent hemorrhage in GSD, and potentially other breeds of dog. Definitive diagnosis of CSS requires specific tests of platelet procoagulant activity.