Qualitative Disorders of platelets

• (2) Inherited platelet-vessel wall interaction:

  •Von-willebrand disease

  •Bernard-soulier syndrome
• (1) aquired platelet vessel wall interaction

  paraproteinemia
• (2) inherited platelet-platelet aggregation interaction :

  •Afibrinogenemia

  •glanzman thrombasthenia
• (1)Aquired platelet-platelet aggregation interaction

  •dysfibrinogenemia
• (3) inherited platelet secretion and signal transduction:

  •storage pool disorders

  •alpha granule deficiency

  •aspirin, NSAIDS
• (1) acquired platelet-secretion and signal transduction

  •aspirin

  •NSAIDS
• ↓ vwf or defect in vwf

  Von Willebrand Disease
• defect in glycoprotein IB/IX/V

  Bernard soulier syndrome
• IgA and IgM coating platelets


  paraproteinemia
• ↓ plasma fibrinogen

  afribrinogenemia
• defect in glycoprotein IIB/IIIa

  Glanzmann thrombasthenia
• abnormal platelet aggregation

  storage pool disorders
• gray platelet syndrome

  alpha-granule deficiency
• abnormality in platelet granules

  gray platelet syndrome
• Dense-granule deficiency

  Chediak-Higashi

  hermansky-pudlak syndrome
• inhibit the cyclooxygenase:

  Aspirin, NSAIDs
• most common cause of congenital bleeding disorders:

  von willebrand disease
• enzyme needed to convert arachidonic acid to TXA2:


  cyclo-oxygenase
• Initial signs and symptoms of von willebrand disease:

  Bleeding from trauma

  Excessive bruising

  Mennorhagia
• lab evaluation for von willebrand disease(3):

  •FVIII activity

  •VWF antigen

  VWF:Ristocetin cofactor
• If lab evaluation to von willebrand disease becomes normal what will you do next (2):

  test for other bleeding disorders

  repeat initial testing in few weeks
• If result is abnormal in lab eval for von willebrand disease, the next thing you will do is (3):

  Ripa aggregation

  collagen binding

  multimer analysis