Hereditary hemorrhagic telangiectasia: systemic therapies, guidelines, and an evolving standard of care

Blood. 2021 Feb 18;137(7):888-895. doi: 10.1182/blood.2020008739.

Abstract

Hereditary hemorrhagic telangiectasia (HHT) management is evolving because of the emergence and development of antiangiogenic therapies to eliminate bleeding telangiectasias and achieve hemostasis. This progress 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.

Publication types

  • Review

MeSH terms

  • Anemia / epidemiology
  • Anemia / etiology
  • Angiogenesis Inhibitors / adverse effects
  • Angiogenesis Inhibitors / therapeutic use
  • Anticoagulants / adverse effects
  • Anticoagulants / therapeutic use
  • Antifibrinolytic Agents / adverse effects
  • Antifibrinolytic Agents / therapeutic use
  • Clinical Trials as Topic
  • Disease Management
  • Epistaxis / drug therapy
  • Epistaxis / etiology
  • Epistaxis / prevention & control
  • Erythrocyte Transfusion
  • Forecasting
  • Gastrointestinal Hemorrhage / drug therapy
  • Gastrointestinal Hemorrhage / etiology
  • Gastrointestinal Hemorrhage / prevention & control
  • Humans
  • Immunologic Factors / therapeutic use
  • Iron Deficiencies
  • Platelet Aggregation Inhibitors / adverse effects
  • Platelet Aggregation Inhibitors / therapeutic use
  • Practice Guidelines as Topic*
  • Prevalence
  • Standard of Care / trends*
  • Telangiectasia, Hereditary Hemorrhagic / complications
  • Telangiectasia, Hereditary Hemorrhagic / therapy*
  • Thrombophilia / chemically induced
  • Thrombophilia / drug therapy
  • Thrombophilia / etiology
  • Tranexamic Acid / therapeutic use

Substances

  • Angiogenesis Inhibitors
  • Anticoagulants
  • Antifibrinolytic Agents
  • Immunologic Factors
  • Platelet Aggregation Inhibitors
  • Tranexamic Acid