Glanzmann’s thrombasthenia is an inherited bleeding disorder that occurs because certain surface proteins on platelets are missing or do not work. It occurs in approximately 1 in 1 million persons. Patients with Glanzmann's thrombasthenia cannot form a platelet plug at sites where the blood vessel was injured, so that the vessel cannot be sealed and bleeding does not stop. Patients often show a higher tendency to bleed from the nose, the gum or during menstruation.
Platelet transfusions have been used most often to treat patients with Glanzmann’s thrombasthenia for bleeding episodes. However, patients may develop antibodies to transfused platelets making future transfusions ineffective. For these patients, recombinant factor VII is a treatment option.
APROM ID# 4497. December 2012