warfarin and Telangiectasia--Hereditary-Hemorrhagic

A 60-year-old man was treated in the hospital for mesenteric vein thrombosis and discharged home on anticoagulation. On warfarin the patient started to bleed profusely from the nose and tongue. He was evaluated by ENT (ears, nose and throat); a nasal endoscopy revealed several vascular ectasias. Subsequent detailed history and general physical examination established the diagnosis of hereditary haemorrhagic telangiectasia also known as Osler-Weber-Rendu syndrome. On further evaluation, pulmonary arteriovenous malformations were diagnosed on imaging and treated by intervention radiology. In hindsight, the diagnosis could have been made in the general practitioner's office with just a routine thorough history and a physical examination at a new patient visit. We report this case to stress upon the importance of vigilant clinical, medical and family history and a thorough examination to establish an early diagnosis of this not-so-rare entity.

Topics: Angiography; Anticoagulants; Combined Modality Therapy; Diagnosis, Differential; Emergency Service, Hospital; Follow-Up Studies; Hemorrhage; Humans; Male; Mesenteric Vascular Occlusion; Mesenteric Veins; Middle Aged; Risk Assessment; Severity of Illness Index; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed; Treatment Outcome; Venous Thrombosis; Warfarin

Hereditary hemorrhagic telangiectasia (HHT) is a rare vascular dysplasia resulting in visceral arteriovenous malformations and smaller mucocutaneous telangiectasia. Most patients experience recurrent nosebleeds and become anemic without iron supplementation. However, thousands may require anticoagulation for conditions such as venous thromboembolism and/or atrial fibrillation. Over decades, tolerance data has been published for almost 200 HHT-affected users of warfarin and heparins, but there are no published data for the newer direct oral anticoagulants (DOACs) in HHT.. To provide such data, a retrospective audit was conducted across the eight HHT centres of the European Reference Network for Rare Multisystemic Vascular Diseases (VASCERN), in Denmark, France, Germany, Italy, the Netherlands and the UK.. Although HHT Centres had not specifically recommended the use of DOACs, 32 treatment episodes had been initiated by other clinicians in 28 patients reviewed at the Centres, at median age 65 years (range 30-84). Indications were for atrial fibrillation (16 treatment episodes) and venous thromboembolism (16 episodes). The 32 treatment episodes used Apixaban (n = 15), Rivaroxaban (n = 14), and Dabigatran (n = 3). HHT nosebleeds increased in severity in 24/32 treatment episodes (75%), leading to treatment discontinuation in 11 (34.4%). Treatment discontinuation was required for 4/15 (26.7%) Apixaban episodes and 7/14 (50%) Rivaroxaban episodes. By a 4 point scale of increasing severity, there was a trend for Rivaroxaban to be associated with a greater bleeding risk both including and excluding patients who had used more than one agent (age-adjusted coefficients 0.61 (95% confidence intervals 0.11, 1.20) and 0.74 (95% confidence intervals 0.12, 1.36) respectively. Associations were maintained after adjustment for gender and treatment indication. Extreme hemorrhagic responses, worse than anything experienced previously, with individual nosebleeds lasting hours requiring hospital admissions, blood transfusions and in all cases treatment discontinuation, occurred in 5/14 (35.7%) Rivaroxaban episodes compared to 3/15 (20%) Apixaban episodes and published rates of ~ 5% for warfarin and heparin.. Currently, conventional heparin and warfarin remain first choice anticoagulants in HHT. If newer anticoagulants are considered, although study numbers are small, at this stage Apixaban appears to be associated with lesser bleeding risk than Rivaroxaban.

Topics: Administration, Oral; Aged; Aged, 80 and over; Anticoagulants; Atrial Fibrillation; Dabigatran; Epistaxis; Female; Humans; Male; Middle Aged; Pulmonary Embolism; Pyrazoles; Pyridones; Retrospective Studies; Rivaroxaban; Telangiectasia, Hereditary Hemorrhagic; Venous Thromboembolism; Warfarin

Spontaneous superior ophthalmic vein thrombosis (SOVT) is a rare entity. We describe three patients with spontaneous ophthalmic vein thrombosis, each with various risk factors.. A retrospective review of three patients with a diagnosis of superior ophthalmic vein thrombosis. Clinical characteristics, radiographic features, management techniques and outcomes are described.. All patients presented with unilateral painful proptosis. Two patients had intact light perception, whereas one patient presented with absent light perception. All patients had identifiable risk factors for thrombosis, which included sickle cell trait, hereditary hemorrhagic telangectasia and colon cancer with recurrent deep vein thrombosis. Anticoagulation was initiated in two patients. Resolution of proptosis was seen in all patients, with no recovery of vision in one patient.. Risk factors for spontaneous superior ophthalmic vein thrombosis are multifactorial. MRI and MRV confirm the diagnosis of SOVT. Despite urgent intervention devastating visual loss may occur.

Topics: Administration, Oral; Adult; Aged; Anticoagulants; Antihypertensive Agents; Colonic Neoplasms; Exophthalmos; Eye; Eye Pain; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Retrospective Studies; Risk Factors; Sickle Cell Trait; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed; Veins; Venous Thrombosis; Visual Acuity; Warfarin

Topics: Anticoagulants; Aspirin; Benzimidazoles; beta-Alanine; Dabigatran; Heparin; Humans; Platelet Aggregation Inhibitors; Telangiectasia, Hereditary Hemorrhagic; Warfarin

Topics: Adult; Aged; Anticoagulants; Antifibrinolytic Agents; Female; Humans; Male; Nuclear Family; Protein S Deficiency; Telangiectasia, Hereditary Hemorrhagic; Tranexamic Acid; Warfarin