Congenital and acquired disorders of coagulation

Abstract

Haemophilia A and haemophilia B are X-linked disorders of blood coagulation, and thus affect males almost exclusively. Acquired coagulation disorders are abnormalities in blood coagulation secondary to a large number of disorders. ‘Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by intravascular activation of coagulation with loss of localization arising from different causes. Coagulopathies secondary to liver disease reflect failure of synthesis of coagulation proteins, poor clearance of activated factors, activation of coagulation and fibrinolytic systems, and loss of haemostatic proteins. Coagulation inhibitors are antibodies that inhibit the function of a specific coagulation factor by neutralizing it, or by promoting its rapid clearance, or by altering the clotting factor. Coagulation abnormalities in cardiopulmonary bypass are multifactorial, resulting in intraoperative and postoperative bleeding. The diagnosis is confirmed when bleeding is rapidly reversed following VK administration and other causes of coagulopathy such as DIC, inherited coagulation disorders, and other causes have been excluded.