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Glanzmann’s thrombasthenia is a rare blood disorder where platelets lack the glycoprotein fibrogen, preventing clotting and causing excessive bleeding. It is inherited and diagnosed through tests, with treatments including hormonal supplements, fibrin sealant medications, and platelet transfusions. Certain medications should be avoided.
Glanzmann’s thrombasthenia (GT) is an extremely rare blood disorder. It affects platelets in the blood. These platelets do not contain a glycoprotein called fibrogen. Without this glycoprotein, platelets cannot bind and form clots that stop bleeding.
Dr. Eduard Glanzmann discovered this platelet abnormality in 1918, when it was called hereditary hemorrhagic thrombasthenia. It is inherited when both parents carry the gene and pass it on to the children and is classified under autosomal recessive disorders affecting blood clotting. Glanzmann’s thrombasthenia causes excessive bleeding from smaller lesions. A child’s lost tooth or paper cut can be extremely dangerous and lead to fatal blood loss.
Platelets have no physical deformities in people who have Glanzmann’s thrombasthenia. Instead, they are normal but lack the ability to engage in clotting. Patients can have either type 1 or type 2. Type 1 occurs when platelets cannot clump or collect and have no ability to retract the clot. People who do not have clumping but have some clot retraction have type 2.
Glanzmann’s thrombasthenia is most commonly seen in early childhood because young children are more prone to cuts and scrapes. Bruising can happen very easily. Frequent nosebleeds and bleeding gums are other common symptoms. Menstruation and childbirth can cause excessive bleeding for women with this disorder.
Glanzmann’s thrombasthenia is diagnosed through tests. Bleeding and clotting times are measured by observing how small cuts stop bleeding and how long it takes. In the laboratory, blood samples are tested with various chemicals to test how platelets react. Samples are also tested to see if glycoproteins are present in the platelets.
Treatments for Glanzmann’s thrombasthenia largely depend on which version of the disorder a patient has. Women may be prescribed hormonal supplements to minimize menstrual bleeding. Other generic medications that can be used for men or women include fibrin sealant medications to help with clotting, iron supplements, and antifibrinolytic medications. Platelet transfusions are often needed for serious injuries to replace lost blood.
For patients who have Glanzmann’s thrombasthenia, certain over-the-counter and prescription medications should be avoided. Blood thinners or medicines that impair clotting should not be taken because they can lead to more bleeding. Nonsteroidal anti-inflammatory drugs should not be taken because they can also interfere with clotting. Doctors must be informed of all medications that have been taken to avoid dangerous interactions or side effects that could make the condition life-threatening.
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