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

Česká verzia

Authors: Zdeněk Adam ¹; Dagmar Brančiková ¹; Gabriela Romanová ²; Luděk Pour ¹; Marta Krejčí ¹; Jiří König ³; Tomáš Nebeský ⁴; Zuzana Adamová ⁵; Martin Štork ¹; Martin Krejčí ¹; Sabina Ševčíková ⁴; Michal Eid ¹; Viera Sandecká ¹; Zdeněk Král ¹
Authors place of work: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Oddělení klinické hematologie FN Brno ²; Oddělní krční, nosní, ušní FN Brno ³; Klinika radiologie a nukleární medicíny – FN Brno ⁴; Ústav patologické fyziologie LF MU a FN Brno ⁵; Chirurgické oddělení nemocnice Vsetín ⁶
Published in the journal: Vnitř Lék 2021; 67(7): 419-424
Category: Review Articles

Summary

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:

sirolimus – bevacizumab – aflibercept – thalidomid – tacrolimus – hereditary hemorrhagic telangiectasia – lenadomid anti‑angiogenic thyrosinkinase inhibitors

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Další literatura u autora a na www.casopisvnitrnilekarstvi.cz