XClose

Institute of Immunity and Transplantation

Home
Menu

Haemophilia

Inherited bleeding disorders are conditions in which the blood does not clot properly. These conditions are passed to a child by one or both parents.

Von Willebrand disease is the most common inherited bleeding disorder, followed by haemophilia A and haemophilia B. Bleeding disorders can also be acquired, which means you are not born with the disorder, but it develops during your lifetime.

The blood clotting process is udually activated by injury to blood vessels. It involves various factors which transform the blood from the liquid state intoa  solid clot that plugs the wound.

People with a defect in some clotting factors may bleed spontaneously or for longer than a healthy person after injury or surgery. Haemophilia A patients are missing or have low levels of clotting factor 8. Haemophilia B patients are missing or have low levels of clotting factor 9. 

About 6,000 people in the UK have haemophilia. Most are males because of the way the condition is inherited. However, females who carry the haemophilia gene may also experience some bleeding problems. A girl could be born with haemophilia if both parents have the haemophilia gene.

Haemophilia can be treated by replacing missing blood clotting factors, which are typically injected into a vein, to help blood clot normally. Such clotting factor replacement therapy can be used to treat bleeding episodes or prevent bleeding.

In addition to preventing severe blood loss, clotting factor replacement therapy can help prevent complications from bleeding such as damage to muscle, joints and organs.

The Royal Free is the premier haemophilia centre in Europe, thanks initially to the pioneering work of Dr Katharine Dormandy from the 1960s. The cloning of the F8 gene performed at the KD haemophilia centre galvanised the production of recombinant clotting factors.

The HD haemophilia centre was the first in Europe to treat haemophilia with recombinant clotting factor and the first in the world to develop successful gene therapy for haemophilia B.

The above images show a transcatheter susbsitute heart valvem (size: 23-29mm). The valve is delivered into the heart through the blood vessels, eliminating the need for open heart surgery and its associated risks.

The Anticoagulation and Thrombosis service, which is part of the HD Haemophilia Centre, is a unique service dedicated solely to the assessment and treatment of blood clots.

Every year, many thousands of pepople in the UK develop a blood clot which is a serious, potentially fatal, medical condition. We work closely with the hospital and the community to provide an individualised clotting risk assessment and treatment plan.