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Basics

Basics

Overview

  • Acquired or hereditary defects that can affect any of the main functions of platelets, including procoagulant activity. Defects are divided in 2 categories: intrinsic and extrinsic (e.g., vWD).
  • Affected animals typically have normal platelet counts but have spontaneous or excessive bleeding; mucosal bleeding is the most common sign.
  • Thrombocytopenic animals with concurrent thrombocytopathia will bleed more excessively than expected for the platelet count.

Signalment

  • Acquired defects are the most common thrombocytopathias seen in companion animals. They occur in all breeds and at all ages.
  • Hereditary platelet function defects may be diagnosed at all ages but may first appear in young animals when excessive bleeding occurs with loss of deciduous teeth.
  • Hereditary defects are rare disorders that have been described in the following breeds/species:
    • Type I Glanzmann thrombasthenia-otter hound and Great Pyrenees
    • Storage pool disease-Chediak-Higashi syndrome-Persian cat; delta-granule-American cocker spaniel; cyclic hematopoiesis-grey collie
    • Thrombocytopathia (CalDAG-GEFI deficiency) -basset hound, spitz, and Landseer
    • Scott syndrome-German shepherd
    • P2Y12 (ADP) receptor mutation-Greater Swiss mountain dog

Signs

  • Often mild spontaneous muco-cutaneous bleeding, such as epistaxis, petecchiation, and gingival bleeding.
  • Prolonged bleeding in some animals during or following diagnostic or surgical procedures.
  • In Scott syndrome, postoperative hemorrhage is the most common sign.

Causes & Risk Factors

Acquired-Drugs

  • NSAIDs (e.g., aspirin) inhibit platelet function by preventing the formation of thromboxane A2. This effect is less pronounced to absent with more selective cyclooxygenase-2 antagonists (e.g., meloxicam, deracoxib).
  • Potentiated sulfonamides and hydroxyethyl starch solutions suppress platelet function in dogs.
  • Penicillins, tetracyclines, anesthetic/sedative agents, and antihistamines cause thrombocytopenia and/or platelet function defects in humans-however, effects have not been documented in dogs and cats.

Secondary to Systemic Disease

Dissemininated intravascular coagulopathy, uremia, anemia, liver disease (cholestasis and acquired or inherited shunts), ehrlichiosis, leishmaniasis, immune-mediated thrombocytopenia, heart disease, and neoplastic disorders (both hematopoietic and non-hematopoietic neoplasms).

Hereditary

  • von Willebrand disease-a deficiency (type I and III) or qualitative defect (type II) of von Willebrand factor.
  • Basset hound, spitz, and Landseer hereditary thrombocytopathia-signal transduction defects due to CalDAG-GEFI mutations.
  • Otter hound and Great Pyrenees with type I Glanzmann thrombasthenia-platelet defect caused by a mutation in glycoprotein IIb-IIIa (integrin Iib3) receptor on the platelet surface.
  • Storage pool disease: Chediak-Higashi syndrome and deficiency in delta-granule storage pool of ADP-aggregation defect caused by lack of adenine nucleotides.
  • Scott syndrome in German shepherd-platelet procoagulant deficiency (failure to externalize phospatidyl-serine on the platelet surface and inability of the platelets to support effective assembly of coagulation complexes).
  • P2Y12 (ADP) receptor mutation in Greater Swiss mountain dog.

Diagnosis

Diagnosis

CBC/Biochemistry/Urinalysis

  • Anemia, if bleeding is severe; regenerative or nonregenerative.
  • Platelet counts typically normal in dogs with inherited thrombocytopathies, but low counts with bizarre and giant platelets seen in some.
  • Biochemical profile-no specific changes.

Other Laboratory Tests

  • Platelet function analyzer-100 and vWF by immune assay-in animals suspected of vWD.
  • Platelet function testing-in specialized laboratories. The most common tests are platelet aggregation and flow cytometry.
  • Coagulation tests (thromboelastography, PT, and APTT)-to eliminate coagulopathy as a cause of hemorrhage; APTT may be prolonged in some animals with von Willebrand disease.
  • Genetic testing of carrier animals.

Diagnostic Procedures

Mucosal bleeding time-to confirm platelet function defect; normal buccal mucosal bleeding time measured using a spring-loaded lancet that makes an incision 5 mm long by 1 mm deep (Triplett, Helena Laboratories, Beaumont, TX) is less than 4–5 minutes in dogs and less than 2–3 minutes in cats.

Treatment

Treatment

Medications

Medications

Drug(s)

  • Desmopressin acetate (DDAVP) (1 µg/kg SC or IV diluted in 20 mL saline administered over 10 minutes) to dogs with von Willebrand disease during a bleeding episode (if effective, effect lasts 2–3 hours).
  • Desmopressin (3 µg/kg SC) improves bleeding time in dogs with thrombocytopathia due to aspirin and liver disease. It is beneficial in many thrombocytopathies in humans, so consider using in dogs with other thrombocytopathias.
  • Give desmopressin to donor 30 minutes before collection of blood for transfusion to dogs with von Willebrand disease or thrombocytopathia.

Follow-Up

Follow-Up

Miscellaneous

Miscellaneous

Abbreviations

  • ADP = adenosine diphosphate
  • APTT = activated partial thromboplastin time
  • DDAVP = 1-desamino-8-d-arginine vasopressin
  • NSAID = nonsteroidal anti-inflammatory drug
  • PT = prothrombin time
  • vWD = von Willebrand disease
  • vWF = von Willebrand factor

Suggested Reading

Boudreaux MK. Inherited platelet disorders. J Vet Emerg Crit Care 2012, 22:3041.

Brooks MB, Catalfamo JL. Platelet dysfunction. In: Bonagura JD, Twedt DC, Kirk's Current Veterinary Therapy XIV. St. Louis, MO: Elsevier, 2009, pp. 292296.

Fry MM. Acquired platelet dysfunction. In: Weiss DJ, Wardrop KJ, Schalm's Veterinary Hematology, 6th ed. Ames, IA: Wiley-Blackwell, 2010, pp. 626632.

Authors Inge Tarnow and Annemarie T. Kristensen

Consulting Editor Alan H. Rebar

Acknowledgment The authors and editors acknowledge the prior contribution of Anthony C.G. Abrams-Ogg.