All::Haematology::Diseases::Antithrombin III deficiency

Intro

What is the inheritance pattern of Antithrombin III deficiency?

autosomal dominant

What are the main clinical features of Antithrombin III deficiency?

  • recurrent venous thromboses
  • arterial thromboses do occur but are uncommon

What is the management of Antithrombin III deficiency?

  • thromboembolic events are treated with lifelong warfarinisation
  • heparinisation during pregnancy*
  • antithrombin III concentrates (often using during surgery or childbirth)

What is the pathophysiology of Antithrombin III deficiency?

  • Antithrombin III deficiency comprises a heterogeneous group of disorders, with some patients having a deficiency of normal antithrombin III whilst others produce abnormal antithrombin III
  • Antithrombin III inhibits several clotting factors, primarily thrombin, factor X and factor IX