Abstract
Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia, Effects on Epistaxis Severity Score and Quality of Life
Highlights
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by telangiectasia and arteriovenous malformations of the skin, mucosal tissues, and internal organs including the gastrointestinal tract, liver, and lungs
We evaluated 6 patients with HHT, calculating their Epistaxis Severity Score (ESS) and performing a quality of life assessment with the 36-Item Short Form Health Survey Questionnaire (SF-36), and we studied the alterations of these evaluations with thalidomide treatment
Anemia is a very common symptom in HHT patients, due to bleeding from telangiectases located in the nasal mucosa and to telangiectases located in the gastrointestinal tract, especially active in older ages
Summary
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by telangiectasia and arteriovenous malformations of the skin, mucosal tissues, and internal organs including the gastrointestinal tract, liver, and lungs. Recurrent epistaxis due to nasal telangiectasia is the most common finding [1,2]. Anemia is a very common symptom in HHT patients, due to bleeding from telangiectases located in the nasal mucosa and to telangiectases located in the gastrointestinal tract, especially active in older ages. The diagnostic criteria for HHT were defined in 2000 and updated in 2011, including epistaxis, telangiectases, vascular malformations, and family history. The presence of 3 of these criteria is suggested to be sufficient for diagnosis [4].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
