A very rare blood disorder due to a blood abnormality.
What is Glanzmann's Thrombasthenia
Glanzmann’s Thrombasthenia (GT) is an abnormality of the platelets. It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is in charge of making the platelets stick together through binding to fibrinogen (clotting factor 1). This means platelets cannot aggregate or clump together to make a plug, hence makes it difficult for the normal blood clot to form.
What are the Symptoms of Glanzmann's Thrombasthenia
The most common types of bleeding seen in patients with Glanzmann’s Thrombasthenia (GT) include:
- Easy bruising
- Petechiae – small red or purple spots on the skin caused by a minor broken capillary
- Bleeding from the nose or gums
- Heavy menstruation (in women)
- Excessive bleeding after surgery or injury
- Excessive bleeding after child birth
Less common types of bleeding seen in patients with Glanzmann’s Thrombasthenia include:
- Bleeding into head and brain (headache, neck ache, drowsiness, loss of consciousness)
- Bleeding into gastrointestinal tract (vomiting blood, bloody or black bowel movements)
- Joint bleeds
In contrast to patients with more common bleeding disorders like congenital hemophilia A or B, patients with Glanzmann’s Thrombasthenia tend to have more mucocutaneous bleeding, or bleeding involving mucous membranes (nose, mouth, stomach) and skin, but do not tend to experience joint or muscle bleeding.
How Does Glanzmann's Thrombasthenia Get Treated?
Most people with platelet function disorders only need treatment during surgical procedures (including dental work) and after injury or accidents. When needed, Glanzmann thrombasthenia may be treated with:
- Antifibrinolytic drugs
Recombinant factor VIIa
Fibrin sealants - Hormonal contraceptives (to control excessive menstrual bleeding)
- Iron replacement (if necessary to treat anemia caused by excessive or prolonged bleeding)
- Platelet transfusions (only if bleeding is severe)
People with inherited platelet function disorders should not take Aspirin®, nonsteroidal anti-inflammatory drugs (such as ibuprofen and naproxen), and blood thinners, which can make their bleeding symptoms worse.
