A Rare Bleeding Disorder: Glanzmann Thrombasthenia

• Published in: International dental journal Vol. 74; p. S246
• Main Authors: Karatepe, Füsun, Gasımlı, Günay, Fahrioğlu, Arman, Kayhan, Kıvanç Bektaş
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.10.2024
• ISSN: 0020-6539
• DOI: 10.1016/j.identj.2024.07.130

Glanzmann Thrombasthenia (GT) is a rare and severe inherited bleeding disorder caused by dysfunction or absence of glycoprotein IIb-IIIa on the platelet membrane. Its occurrence rate is approximately 1 in 1.000.000 individuals, yet it tends to be more prevalent in communities with higher rates of consanguinity. The most common clinical presentations are unexplained mucocutaneous bleeding, purpura, epistaxis, gum bleeding and menorrhagia. During surgical procedures, a prolonged bleeding time and the absence of platelet aggregation on peripheral smears suggest Glanzmann Thrombasthenia. Two young women patients, sisters, referred to our Department of Oral and Maxillofacial Surgery for tooth extraction. Both sisters have the same bleeding disorder, Glanzmann thrombastenia (GT). After intraoral and radiological evaluation their treatment plans were formed which included dental extraction in both cases. The consultation with the hematology department resulted in the decision to proceed with platelet transfusion and the use of antifibrinolytic drugs. Extractions in both cases were done in atraumatic and careful approach and both healed uneventfully. Without any serious complications of bleeding. In patients with GT, early diagnosis and performing surgical procedures under appropriate precautions can help prevent bleeding complications. Surgical procedure can be safely performed with collaboration with a hematologist. Platelet transfusion is the standard treatment during the perioperative and postoperative periods for patients with GT. The management of our two cases indicates that proper application of a comprehensive transfusion protocol may prevent excessive postoperative bleeding. GT can be a severe bleeding disorder, however, an excellent prognosis can be achieved with careful supportive care.