Perioperative administration of recombinant activated factor VII in a Glanzmann's thrombasthenia patient with platelet refractoriness: case report

Abstract Glanzmannʼs Trombasthenia (GT) is a genetic disorder, that develops with a tendency toward bleeding and is characterized by the absence or decrease in platelet aggregation. Surgical bleeding may be difficult to control. Platelet transfusion is the main treatment, albeit refractoriness can occur. We describe the case of a patient with GT and platelet refractoriness, who was submitted to radical prostatectomy and dental extraction. The perioperative treatment with apheresis platelet concentrate and activated recombinant factor seven allowed the procedures to be performed uneventfully. We discuss the complexity of the case and the treatment option.

Glanzmann's thrombasthenia and pregnancy

Glanzmann's thrombasthenia is rare autosomal recessive platelet disorder caused by abnormal platelet glycoprotein complex [GP IIb-IIIa] leading to excessive mucosal bleeding. Patients with Glanzmann's thrombasthenia may present with hemorrhagic symptoms including mucosal bleeding, easy bruising and post-surgical bleeding. We report a 37-year old lady, who underwent a cesarean section at term. She received recombinant factor VIIa at the time of surgery. She had uneventful post operative period and she was discharged home in good general conditions