Glanzmann thrombasthenia (GT) is a rare genetic platelet disorder in which the platelets have qualitative or quantitative deficiencies of the fibrinogen receptor αIIbβ3. The signs and symptoms of GT include the following:
• Mucosal bleeding
• Gingival bleeding
• Petechiae and ecchymoses
• Gastrointestinal bleeding
HOW IS GLANZMANN THROMBASTHENIA DIAGNOSED?
A general physician diagnoses GT by using the following tests:
1. Complete blood cell count
2. Prothrombin time and activated partial thromboplastin time.
3. 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?
In women with this condition, hormonal therapy may be needed to suppress menstrual bleeding.