Clin Pediatr Hematol Oncol.
2014 Apr;21(1):29-32.
A Case of Glanzmann's Thrombasthenia Successfully Managed after Tonsillectomy
- Affiliations
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- 1Department of Otorhinolaryngology-Head & Neck Surgery, Inha University School of Medicine, Incheon, Korea.
- 2Department of Pediatrics, Inha University School of Medicine, Incheon, Korea. pedkim@inha.ac.kr
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.
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