Chapter

Recombinant Protein Drugs

Part of the series Milestones in Drug Therapy pp 67-88

Clotting factors VIII and IX

  • George G. BrownleeAffiliated withChemical Pathology Unit, Sir William Dunn School of Pathology, University of Oxford
  • , Paul L. F. GiangrandeAffiliated withOxford Haemophilia Centre, The Churchill Hospital

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Abstract

Haemophilia is the most common congenital disorder of coagulation and affects approximately 1 in 10,000 males around the world. Haemophilia A is due to a deficiency of factor VIII in the circulating blood whilst haemophilia B (also known as Christmas disease) is a clinically identical disorder caused by factor IX deficiency. It is less common than haemophilia A and affects 1 in about 30,000 males. Both factors VIII and IX are essential glycoproteins in the clotting cascade [1] (Fig. 1). The hallmark of severe haemophilia is recurrent and spontaneous haemarthrosis, typically affecting the hinge joints such as the ankle, knee and elbow. The severity of bleeding depends upon the level of factor in the blood.