Vnitr Lek 2021, 67(7):419-424 | DOI: 10.36290/vnl.2021.110

Hereditary hemorrhagic telangiectasia (Osler‑Weber‑Rendu syndrome) - Part II. Pharmacological therapy and international guidelines for the therapy 2020

Zdeněk Adam1, Dagmar Brančíková1, Gabriela Romanová2, Luděk Pour1, Marta Krejčí1, Jiří König3, Tomáš Nebeský4, Zuzana Adamová5, Martin Štork1, Martin Krejčí1, Sabina Ševčíková4, Michal Eid1, Viera Sandecká1, Zdeněk Král1
1 Interní hematologická a onkologická klinika LF MU a FN Brno
2 Oddělení klinické hematologie FN Brno
3 Oddělní krční, nosní, ušní FN Brno
4 Klinika radiologie a nukleární medicíny - FN Brno
5 Ústav patologické fyziologie LF MU a FN Brno
6 Chirurgické oddělení nemocnice Vsetín

Hereditary hemorrhagic telangiectasia also known as Osler-Weber-Rendu syndrome, is an disorder that causes abnormal blood vessel formation with bleeding. Inhibition of angiogenesis amelioretes bleeding complication. Anti-angiogenic agents such as bevacizumab, aflibercept, thalidomid, lenadomid and other new anti-angiogenic thyrosinkinase inhibitors, as well as sirolimus and takrolimus have emerged as a promising systemic or local therapy in reducing bleeding complications but are not curative. Other pharmacological agents include iron supplementation, antifibrinolytics and hormonal treatment. This review concentrates on new anti-agioproliferative drugs with effect in HHT- discusses the new biology of HHT, management issues that face the practising hematologist, and considerations of future directions in HHT treatment.

Keywords: hereditary hemorrhagic telangiectasia, bevacizumab, aflibercept, thalidomid, lenadomid anti‑angiogenic thyrosinkinase inhibitors, sirolimus, tacrolimus.

Published: November 1, 2021  Show citation

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Adam Z, Brančíková D, Romanová G, Pour L, Krejčí M, König J, et al.. Hereditary hemorrhagic telangiectasia (Osler‑Weber‑Rendu syndrome) - Part II. Pharmacological therapy and international guidelines for the therapy 2020. Vnitr Lek. 2021;67(7):419-424. doi: 10.36290/vnl.2021.110.
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