Glanzmann thrombasthenia was first described by Dr. Eduard Glanzmann in 1918. Predominant in ethnic population, it is an inherited blood clotting disorder. The probability of death following bleeding is estimated at approximately 5-10%.The current article describes pathophysiology, clinical presentation, management and prevention of the disorder. Glanzmann thrombasthenia (GT) is mainly caused by a deficiency of glycoprotein IIb/IIIa which is present on the surface of platelets.
The genes of both these proteins are present on chromosome 17 and 50% activity of each protein is enough to support normal platelet aggregation. Because of this protein deficiency, platelets fail to aggregate and form a plug causing bleeding. Females are slightly more prone to GT than males. Patients with GT experiences an episode of mucocutaneous bleeding at birth or in early infancy. GT is clinically significant…
