Bleeding disorders due to abnormal platelet function

Bleeding disorders due to abnormal platelet function
Updated: 08/12/2020
© Jun Wang, MD, PhD

General features
  • Inherited or acquired
  • Three large categories
Defects of adhesion
Defects of aggregation
Disorders of platelet secretion
Glanzmann’s disease
  • Qualitative or quantitative deficiencies of the fibrinogen receptor GPIIb/IIIa
  • Platelet type bleeding of mucosa, skin, etc
  • Management: Avoidance of medications that affect platelet function, treatments of anemia, etc
  • Platelet transfusion for bleeding patients
  • Recombinant Factor VIIa may be considered
Bernard-Soulier Syndrome
  • Group of hereditary platelet disorders characterized by
Thrombocytopenia
Giant platelets
Qualitative platelet defects
  • Absence or decreased expression of the GPIb/IX/V complex, a receptor for von Willebrand factor, on the surface of the platelets
  • Autosomal recessive
  • Clinical presentations: Easy bruising, bleeding
  • Treatment: Supportive, may consider DDAVP, or similar approach to Glanzmann’s Disease
Other acquired platelet function disorders
  • Primary cause are anti-platelet drugs
Aspirin
NSAIDs
Dipyramidole
Clopidigrel
GP IIb / IIIa receptor antagonists



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