Glanzmann's disease

Glanzmann's disease

Glanzmann's thrombasthenia Hematology An AR inborn error of metabolism characterized by coagulation defects Clinical Epistaxis, bruising, excess bleeding Lab ↑ Bleeding time, defective clot retraction, ↓ platelet aggregation with ADP, collagen, thrombin Management Platelet transfusions; avoid antiplatelet agents–eg, aspirin, NSAIDs