Background: Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history.
Aims: Venous thromboembolic disease is a poor prognostic factor in this disease given the risk of increased bleeding caused by anticoagulant therapy.
Methods: We report a new case of a 56-year-old patient with Osler disease who developed recurrent thromboembolic venous disease .
Results: A 56-year-old patient was hospitalized in June 2016 for treatment of deep vein thrombosis of the lower limb complicated by bilateral proximal pulmonary embolism. There was no notion of sedentarity or trauma. The patient reported the notion of recurrent epistaxis from a young age. He did not report any notion of hemoptysis or digestive bleeding. On clinical examination, telangiectasias were observed on the face, the inner sides of the cheeks and on the hands. These lesions bled at the slightest touch.
On biology, the blood count, hemostasis, renal, hepatic assessments were without notable abnormality.
The dosage of coagulation proteins and Circulating anticoagulants and anticardiolipin antibodies without abnormalities.
The diagnosis of Rendu Osler disease was made by the association of recurrent epistaxis and multiple telangiectasias. There was no similar case in the family. There were no other associated visceral lesions. The thoraco-abdominal CT angiography as well as the echocardiography were without abnormalities. The genetic study was not practiced given the unavailability in our hospital. Under anticoagulant treatment “acenocoumarol”, the patient reported a worsening of epistaxis . This anticoagulant treatment was then stopped after 6 months. Two months later, the patient presented with a recurrence of DVT complicated by pulmonary embolism which required a resumption of anticoagulant treatment for a prolonged period given the recurrent nature of the the venous thromboembolic disease.
Conclusion(s): According to a review of the literature, this association does not appear to be fortuitous and is a factor of disease severity.
To cite this abstract in AMA style:Ben Salah R, Ben Mefteh A, Dammak c, Frikha F, bAHLOUL Z. Rendu-Osler disease and recurrent thromboembolic venous disease [abstract]. https://abstracts.isth.org/abstract/rendu-osler-disease-and-recurrent-thromboembolic-venous-disease/. Accessed February 28, 2024.
« Back to ISTH 2022 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/rendu-osler-disease-and-recurrent-thromboembolic-venous-disease/