Von Willebrand Disease (hereditary Platelet Dysfxn)

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  • Von Willebrand Disease (hereditary platelet dysfxn)

Von Willebrand Disease (hereditary platelet dysfxn) Detail 🌳 View as Mindmap

Etiology

  • Autosomal dominant disorder of platelet aggregation
  • Due to abnormally low levels of Von Willebrand's factor (protein modulating platelet adhesion) and/or qualitative issues impairing VFW factor function
  • Acquired VwD less common and is associated with processes that disrupt Vw Factor function: mechanical forces (abnormal valves, VSDs, LVADs, other), meds (e.g. cipro, valproate), malignancies (myeloma, mugs, myeloproliferative d/o), metabolic disorders (hypothyroidism), other

Risk Factors

  • Family history of bleeding disorder

Symptoms

  • Variable severity and age of presentation based on amount of Von Willebrand's factor produced
  • Spontaneous mucocutanous bleeding → gums, nosebleeds
  • GI bleeding from angiodysplasia (higher shear forces in these vessels unmasks the disease)
  • Excessive bleeding during/after surgical procedures, minor trauma, dental extraction
  • Hematomas and occasionally intra-articular bleeding w/severe disease
  • In women, menorrhagia a common presenting symptom

Physical Exam Findings

  • Petechiae
  • Spontaneous ecchymoses, mucosal bleeding
  • Excessive bleeding after minor trauma

Tests

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