Hereditary hemorrhagic telangiectasia (OslerWeberRendu syndrome) - Part II. Pharmacological therapy and international guidelines for the therapy 2020.

ABSTRACT

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.