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Glanzmann Thrombasthenia – A Rare but Important Platelet Disorder – Iranian Comprehensive Hemophilia Care Center
ICHCC/LinkedIn
Jun 8, 2026, 12:02

Glanzmann Thrombasthenia – A Rare but Important Platelet Disorder – Iranian Comprehensive Hemophilia Care Center

Iranian Comprehensive Hemophilia Care Center  (ICHCC)  shared a post on LinkedIn:

“Glanzmann Thrombasthenia: A Rare but Important Platelet Disorder

Glanzmann Thrombasthenia (GT) is a rare inherited platelet function disorder caused by deficiency or dysfunction of the platelet glycoprotein IIb/IIIa (αIIbβ3 integrin), resulting in impaired platelet aggregation.

Key Clinical Features:

  • Recurrent epistaxis
  • Easy bruising
  • Gingival bleeding
  • Menorrhagia
  • Excessive bleeding after surgery or dental procedures

Laboratory Findings:

  • Normal platelet count and morphology
  • Normal PT and aPTT
  • Prolonged bleeding time or abnormal PFA
  • Absent platelet aggregation with ADP, collagen, arachidonic acid, and epinephrine
  • Normal agglutination with ristocetin

Diagnosis:

  • Platelet aggregation studies
  • Flow cytometry for CD41/CD61 expression
  • Molecular testing of ITGA2B and ITGB3 genes

Management:

  • Local hemostatic measures
  • Antifibrinolytic agents (tranexamic acid)
  • Platelet transfusion for major bleeding
  • Recombinant factor VIIa in refractory cases or platelet alloimmunization
  • Genetic counseling

Diagnostic Pearl:
‘Normal ristocetin response with absent aggregation to all other agonists’ is the classic laboratory signature of Glanzmann Thrombasthenia.”

Iranian Comprehensive Hemophilia Care Center

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