Resposta terapêutica da talidomida na telangiectasia hemorrágica hereditária (síndrome de Osler-Weber- Rendu): revisão sistemática.

Abstract

Hereditary Hemorrhagic Telangiectasia (HHT) or Osler-Weber-Rendu Syndrome is a rare systemic fibrovascular dysplasia characterized by spontaneous and recurrent bleeding. Because it is a pathology of difficult treatment, all therapeutic modalities are palliative. In this context, further studies on the use of thalidomide in this hemorrhagic disorder bring a new perspective to improve the quality of life of the patients. Objectives: To evaluate the therapeutic response and adverse effects of thalidomide on gastrointestinal bleeding and epistaxis in HHT. Methods: A bibliographic survey in PUBMED and BVS/BIREME databases, of articles published between the years 2010 to 2015, in english, spanish, portuguese and italian languages, using the keywords: "Thalidomide", "Hereditary Hemorrhagic

telangiectasia" and "Osler-Weber-Rendu Syndrome". Results: After performing pre- selection of the articles by reading titles and abstracts, 12 were selected; of these,

remained in the review 8 articles. Discussion: The studies are generally concordant about the potential benefits of thalidomide, which has been shown, in patients with HHT, be effective in reducing the frequency and intensity of nosebleeds and gastrointestinal bleeding, in improving hematological profile and reduce the need for blood transfusions, as also noted the worsening of clinical condition and increased need for blood transfusions after discontinuation of therapy. Conclusion: Thalidomide appears to be a potential candidate for the treatment of severe epistaxis associated with HHT unresponsive to conventional therapies and for refractory gastrointestinal bleeding secondary to angiodysplasia. Future researches are needed and should be directed to identify the lowest effective dose of thalidomide.