Hereditary hemorrhagic telangiectasia (HHT) management is evolving due to the emergence and development of antiangiogenic therapies to eliminate bleeding telangiectasias and achieve hemostasis. This is reflected in recent clinical recommendations published in the Second International Guidelines for the Diagnosis and Treatment of HHT, in which systemic therapies including antiangiogenics and antifibrinolytics are now recommended as standard treatment options for bleeding. This review highlights the new recommendations especially relevant to hematologists in managing bleeding, anticoagulation, and anemia in patients with HHT.

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