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Genetic counselling may help the affected individuals and their families.
Glanzmann Thrombasthenia is a medical condition characterized by improper clotting of blood. It is a congenital hemorrhagic disorder that is caused by improper levels of glycoprotein. Without enough glycoprotein platelets do not stick together to arrest the bleeding. Symptoms are:
• Frequent nosebleeds
• Easy bruising
• Bleeding gums
• Heavy menstrual bleeding
• Bleeding during or after surgery
HOW IS GLANZMANN THROMBASTHENIA DIAGNOSED?
People affected with this condition have a normal count of platelets but have a prolonged bleeding time. The condition is diagnosed by tests that determine if there is a deficiency of the aIIbb3 (GPIIb/GPIIIa) receptor. These tests usually involve monoclonal antibodies and flow cytometry.
HOW IS GLANZMANN THROMBASTHENIA TREATED?
Treatment of GT includes preventive measures such as avoidance of antiplatelet agents (e.g., aspirin and NSAIDs), iron or folate supplementation for anemia, and vaccination for hepatitis B due to the infectious risks associated with multiple transfusions. Patients with GT who are bleeding require platelet transfusion. Recombinant coagulation factor VIIa was approved by the FDA in July 2014 for bleeding episodes and perioperative management in patients with GT refractory to platelet transfusions, with or without antibodies to platelets.
DID YOU KNOW?
The disorder was first described by Dr. Eduard Glanzmann in 1918.