Glanzmann thrombasthenia (GT) is a rare inherited disorder associated with abnormal platelet function. Non-surgical bleeding is common and can be life-threatening. A 70-year-old woman with GT presented with a 3 week history of melena and asthenia. She had hemoglobin of 3.8 g/dL. Both upper and lower endoscopies were inconclusive. Video capsule endoscopy showed multiple angioectasias and erosions in the distal ileum. Following several days of recurring intestinal hemorrhage, only treatment with recombinant activated factor VII proved to be effective, enabling patient stabilization with no further bleeding. Control and prevention of bleeding among these patients is imperative and remains challenging.
Learning points: Glanzmann thrombasthenia is a rare inherited bleeding disorder of platelet function caused by a quantitative or qualitative defect of the platelet membrane glycoprotein (GP) IIb/IIIa (integrin αIIbβ3) complex.Bleeding is a concern in patients with GT due to its potential risk of severity and uncontrollability. It also greatly impacts on the quality of life of the patient.Recombinant FVIIa has been reported to be a safe and effective hemostatic agent when bleeding is not responsive to local management, antifibrinolytics or platelet transfusion.
Keywords: Glanzmann thrombasthenia; bleeding; rFVIIa.