Glanzmann Thrombasthenia

Glanzmann Thrombasthenia
It affects the ability of the blood platelets to gather around the site of a broken blood vessel. The platelets are sometimes present in normal quantity but are unable to work normally. This protein is needed so that platelets aggregate around an injury to a blood vessel. Because of the deficiency, platelets fail to form a plug to stop the bleeding. Glanzmann Thrombasthenia has three categories of severity. Type I (Severe) is an individual with level less than 5% of normal. Type II (Less severe) is an individual with between 5% and 20% of normal and Type III (Least severe)is a variant of Thrombasthenia with levels of more than 50% of normal, but with major abnormalities in the way platelets aggregate.

What are the symptoms?
People with Glanzmann Thrombasthenia have symptoms:
Common symptoms
• Nosebleeds
• Bruising
• Gastrointestinal bleeding
• Increased and longer menstrual bleeding

Other symptoms
• Spontaneous abortion in the first quarter
• Bleeding during and after Surgery or Trauma

How is it diagnosed?
Glanzmann Thrombasthenia deficiency is diagnosed by a variety of blood tests that should be performed by a specialist at a Haemophilia/bleeding disorders treatment centre.
How is it treated?
Prolonged pressure at the site of a cut on the skin or in the mouth and packing in the nose in the event of a nose bleeds (epistaxis). Tranexamic acid is used to stop clots from being broken down. In the case of severe bleeds intravenous transfusion of platelets may be required. This is treatment is limited by the appearance of antibodies that destroy the transfused platelets. Recombinant Factor VIIa may also be used in certain cases.

Factor concentrates
Recombinant VIIa are also available. They are made in the laboratory and not from human plasma, so they carry no risk of infectious disease. Factor concentrates are administered intravenously.

Platelet transfusions
Platelets are small blood cells that are involved in the formation of blood clots and the repair of damaged blood vessels. Certain clotting factors, including factor V, are stored in small sacs inside them. Platelet transfusions are sometimes used to treat factor V deficiency.
Tranexamic Acid
Tranexamic acid and aminocaproic acid are used to hold a clot in place in certain parts of the body, such as the mouth, bladder, and uterus. They are also very useful in many situations, such as during dental work, but are not effective for major internal bleeding or surgery. They are also used to help control excessive menstrual bleeding. Antifibrinolytic drugs can be administered orally or by injection.

Hormonal therapies
Hormonal contraceptives (birth control pills) help control menstrual bleeding (read more).