Double jeopardy, glomangiopericytoma and Glanzmann thrombasthenia resulting in recurrent epistaxis: a case report.

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.

Acquired FVII Deficiency and Acute Myeloid Leukemia: A Case Report and Literature Review.

Factor VII (FVII) deficiency is the most common among all rare inherited bleeding disorders. However, acquired FVII deficiency (aFVIID) is uncommon. Only few cases in the literature have been reported. Herein, we present a case of an aFVIID associated with acute myeloid leukemia (AML), along with a literature review regarding this condition. A 50 year old Arab male patient was diagnosed with AML at the hematology department of our institution. At admission, coagulation tests showed a prolonged prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) and a slightly elevated fibrinogen level. Prothrombin complex coagulation factors dosing (PCCFD) revealed a decrease only in FVII levels. The patient, however, did not experience any bleeding. The evolution of the health of the patient was marked by a normalization of PT and FVII levels and complete remission.