ISSN: 1300-7777 E-ISSN: 1308-5263
Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia, Effects on Epistaxis Severity Score and Quality of Life [Turk J Hematol]
Turk J Hematol. Ahead of Print: TJH-65390 | DOI: 10.4274/tjh.2018.0190  

Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia, Effects on Epistaxis Severity Score and Quality of Life

Mehmet Baysal, Elif G Ümit, Hakkı Onur Kırkızlar, Ali Caner Özdöver, Ahmet Muzaffer Demir
Department of Hematology, Faculty of Medicine, Edirne, Turkey

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominantly inherited disorder characterized with bleeding episodes. These episodes tend to happen spontaneously and reduce the quality of life. Patients are often unresponsive to local measures. With the pathophysiological role of angiogenesis in HHT, antiangiogenic drugs including thalidomide were used to control bleeding episodes. In our study, we evaluated 6 patients with HHT, evaluated them with epistaxis severity score (ESS) and performed a quality of life assessment with 36 Item Short Form Questionnaire (SF-36) and the alterations of these evaluations with thalidomide treatment. Three patients were male and three were female. Mean age was 60.50 years. No side effects were observed during the treatment period. Improvements of certain SF-36 dimensions including physical functioning, physical component summary and mental component summary and ESS were observed after the treatment. Thalidomide may be effective to control the bleeding episodes and with a reasonable tolerance profile in patients with HHT.

Keywords: Hereditary hemorrhagic telangiectasia, Thalidomide, Epistaxis, Quality of life




Corresponding Author: Mehmet Baysal, Türkiye


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Impact Factor (2016) = 0.686