Glanzmann’s thrombasthenia (GT) is an inherited autosomal disorder that begins at birth. It causes prolonged or spontaneous bleeding and affects one out of one million people.
Patients tend to bruise easily and may have frequent nose bleeds and bleeding gums. When bleeding occurs just beneath the skin, purple marks appear. In some cases, pooled blood forms a hematoma. Women with this disorder can have extremely heavy periods, and are subject to severe blood loss during pregnancy and childbirth.
As people with Glanzmann thrombasthenia get older, spontaneous bleeding becomes less frequent. The severity varies even among members of the same family.
Treatment is based around preventive actions, including the avoidance of antiplatelet agents like aspirin. Patients usually take iron supplements for anemia and are vaccinated for hepatitis B as a precaution. This is because of the risks associated with multiple blood and platelet transfusions, which are required when a patient is bleeding.
On July 7, 2014, the US Food and Drug Administration (FDA) approved NovoSeven RT, a recombinant factor VIIa product manufactured by NovoNordisk, as the first recombinant treatment for GT. This was good news for the GT community, because prior to the approval of NovoSeven RT, there were no other treatments except direct platelet transfusions.
If you would like to learn more about GT, contact the Glanzmann’s Research Foundation or read more here.
