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.”
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