Glanzmann Thrombasthenia

• Normal number of platelets, but abnorml function
• Missing the protein that binds fibrinogen to platelets
• SEVERE mucous membrane and postop bleeding, with neonatal purpura
• have prolonged bleeding time, with normal PT/PTT
• Splenectomy, IVIG, steroids don’t help

also see Platelet disorders, Bernard-Soulier Syndrome