Fiche publication
Date publication
décembre 2023
Journal
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis
Auteurs
Membres identifiés du Cancéropôle Est :
Pr DEBRY Christian
Tous les auteurs :
Hammami E, Fath L, Debry C, Desprez D
Lien Pubmed
Résumé
Glanzmann thrombasthenia is a rare bleeding disorder induced by inherited defects of the platelet membrane αIIbβ3 glycoprotein. Glomangiopericytoma, on the other hand, is a very rare sinonasal tumor demonstrating a perivascular myoid phenotype. We herein report the first described case in the literature of Glanzmann thrombasthenia and glomangiopericytoma. The patient is a 40-year-old man diagnosed with type 1 Glanzmann thrombasthenia who presented with repetitive and profuse posterior epistaxis initially managed with platelet transfusions and recombinant activated factor VII (rFVIIa). Due to the unresolved epistaxis, nasal endoscopy was performed revealing a vascularized tumor. Subsequently, a sphenopalatine artery embolization followed by a surgical excision of the tumor was performed. The pathology report diagnosis of the tumor was glomangiopericytoma. This case sheds the lights on a very rare cause of epistaxis in a patient with Glanzmann thrombasthenia, with a challenging multidisciplinary management. A local cause of epistaxis should always be considered even in case of a diagnosed bleeding disorder, especially when the bleeding is recurrent.
Référence
Blood Coagul Fibrinolysis. 2023 12 21;:
