Glanzmann Thrombasthenia

  • Impaired platelet aggregation from genetic deficiency of GpIIb/IIIa
  • Autosomal recessive inheritance
  • GpIIb/IIIa (a platelet integrin) → Defect in platelet plug formation
  • Bleeding from mucous membranes
  • Epistaxis, petechiae/purpura
  • Bleeding time
  • Normal PT/PTT
  • Normal platelet count and morphology
  • Platelet transfusions with HLA-matched and leukocyte depleted blood products
  • Supportive care (iron and folate supplementation)
  • Bernard-Soulier (↓ platelet count, ↑ bleeding time, and ↓ GpIb)
  • ITP (↓ platelet count, ↑ bleeding time, and anti-GpIIb/IIIa antibodies)
  • TTP (↓ platelet count, ↑ bleeding time, and defet/deficient ADAMTS 13)