A Case of Glanzmann's Thrombasthenia Successfully Managed after Tonsillectomy / 임상소아혈액종양

ABSTRACT

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disease and platelet function disorder, in which platelet membrane GP IIb/ IIIa complex is defective and platelet aggregation is undeveloped. GT is characterized by mucocutaneous hemorrhages, such as, epistaxis, purpura, gingival bleeding, and menorrhagia, severe bleeding complications during surgery. We report the case of a 6-year-old boy with GT who underwent tonsillectomy. Here, we focus on perioperative hemostatic management using recombinant factor VIIa, fibrin glue and hemostat materials.