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Year: 2013  Vol. 17   Num. Suppl. 1  - Print:
TROMBASTENIA DE GLANZMANN: CASE REPORT
Author(s):
Ludmila Helene Ferreira de Freitas, Fabio Rezende Pinna, Nelson Almeida D'Ávila Melo, Richard Louis Voegels, Samanta Marques Reis e Silva
Abstract:

OBJECTIVE: To report a case of epistaxis in a child with Glanzmann's thrombasthenia. CASE REPORT: a 9-year-old Caucasian girl presented in the otorhinolaryngology emergency department with profuse, sudden-onset bleeding in the left anterior nostril, without any previous history of trauma. She underwent anterior-posterior nasal packing with no more bleeding after the procedure. This was her third episode of spontaneous non-traumatic epistaxis that needed packing. Additionally, she had a history of bleeding after minimal trauma and gingivorrhagia. Platelets and coagulation tests including fibrinogen, Von Willebrand factor, activated partial thromboplastin time, thrombin time and prothrombin time were normal. She looked pale, her heart rate before the packing was 160 beats per minute, blood pressure was 90/50 mmHg and hemoglobin was 4.4 g%. Because of the severity of the case, the patient underwent ligation of the sphenopalatine artery, but despite correct identification of the artery and cauterization of its major branches, the bleeding did not stop. Therefore, it was decided that the surgery should be stopped and nasal packing resumed. A platelet aggregation test was performed and there was no aggregation with natural platelets agonists (ADP, collagen or amino acids), there was aggregation with ristocetin, which led us to diagnose Glanzmann's thrombasthenia. The patient received a platelets infusion and the packing was removed in the operating room, with no subsequent bleeding. CONCLUSION: Hematological disorders should be considered in patients with recurrent bleeding problems, even if routine hematological tests are normal.

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