Information About Glanzmann Thrombasthenia

Jun 28, 2022

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Platelets play an important role in many restorative physiological processes, including hemostasis. During hemostasis, damaged subendothelial cells release adhesive proteins (such as collagen and thromboplastin) and fibrinogen, which bind to aggregated platelets at the site of injury and form a platelet plug. Platelets then serve as a surface and phospholipid source for coagulation cofactor attachment. Following activation of the coagulation pathways, fibrin attaches to activated platelets, resulting in the formation of a thrombus. Any disruption in platelet function, whether acquired or inherited, causes bleeding. Glanzmann's thrombasthenia (GT) is an uncommon clotting disorder caused by impaired platelet function . Eduard Glanzmann described Glanzmann thrombasthenia (GT) in 1918 after discovering a functional abnormality of platelets with impaired clot retraction. It is a rare genetic platelet disorder affecting the platelet glycoprotein IIb/IIIa (GPIIb/IIIa) complex, with a one in one million occurrence. Integrin IIb3, formerly known as GPIIb/IIIa, is a fibrinogen receptor on platelets. When platelets are activated, it changes to an active state to allow for fibrinogen binding. Platelets accumulate and provide primary haemostasis as they bind fibrinogen. Bleeding can occur spontaneously or as a result of an injury that lacks functioning fibrinogen receptors or a sufficient number of them. Platelets in GT are less effective at producing thrombin, which is required for the conversion of fibrinogen to fibrin . In patients, GTs are classified into three types: type I, type II, and variations. Patients with Type I have 5% of the average amount of GPIIb/IIIa; patients with Type II have 5-20% of the average amount of GPIIb/IIIa; and patients with variant types have one-half of the average amount of GPIIb/IIIa. GT, like many other bleeding disorders, is treated in a multi-tiered fashion. Local pressure, cauterisation, sutures, or ice therapy may be used as initial treatment for minor bleeding episodes. Platelet transfusion is commonly used in GT for surgical prophylaxis and treatment of moderate to severe bleeding. Many patients benefit from both preventative and symptomatic care. Curative treatment with haematopoietic stem cell transplant has been shown to be effective in a small number of GT patients with extremely poor quality of life.

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