Double jeopardy, glomangiopericytoma and Glanzmann thrombasthenia resulting in recurrent epistaxis: a case report.
Blood Coagul Fibrinolysis
; 35(2): 62-65, 2024 Mar 01.
Article
in En
| MEDLINE
| ID: mdl-38179703
ABSTRACT
Glanzmann thrombasthenia is a rare bleeding disorder induced by inherited defects of the platelet membrane αIIbß3 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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Thrombasthenia
/
Head and Neck Neoplasms
Type of study:
Diagnostic_studies
/
Etiology_studies
Limits:
Adult
/
Humans
/
Male
Language:
En
Journal:
Blood Coagul Fibrinolysis
/
Blood coagul. fibrinolysis
/
Blood coagulation and fibrinolysis
Journal subject:
ANGIOLOGIA
/
HEMATOLOGIA
Year:
2024
Document type:
Article
Country of publication:
Reino Unido