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Auckland DHB Haemophilia Centre

Public Service, Haematology

Haemophilia and other Bleeding Disorders

What is Haemophilia?

Haemophilia is a life-long blood clotting disorder which, in most cases, affects boys. It is a genetic condition.

There are two common forms:

  • Haemophilia A (problems that affect clotting Factor Eight)
  • Haemophilia B (problems that affect clotting Factor Nine)

These forms involve bleeding into the muscles and joints, which can lead to rapid damage and long-term disability.

These forms of bleeding disorder are classed as mild, moderate or severe, depending on the amount of Factor 8 or 9 in your body.

Because this is a genetic condition, you may have more than one family/ whānau member affected. Some people may have several joints affected by changes in response to bleeding, particularly where this has occurred in childhood.

It is thought that around 85% of all bleeding episodes occur within the muscles and joints of the arms and legs. Most of these episodes occur within the hinge joints at the knees, ankles and elbows. Joints affected by repeated bleeding episodes are called 'target' joints.


Other Bleeding Disorders
It is important to remember that other types of bleeding disorder, such as Von Willebrand’s Disorder, are treated by the Auckland Haemophilia Centre.

Rarer bleeding disorders such as Factor 7, 10 or 13 deficiencies, platelet and fibrin disorders are also managed by the Centre.

These disorders may affect women and men. They are not usually associated with muscle and joint bleeding except in very severe cases.


More Information about Joint Bleeds
Joint bleeds are called haemarthroses. These occur as a result of trapping and tearing of the membrane that lines the joint. This membrane is called the synovium. The synovium is a structure which normally creates the nourishing, lubricating fluid inside our joints. When this membrane is damaged, blood fills the joint. A diagram of this can be found by clicking this link.

If the bleeding into the joint is not treated quickly, iron from the blood starts to affect both the cartilage and the synovium itself. Cartilage is a very strong structure that acts like a shock absorber on the surfaces of our joints. Iron from blood reduces the stability of cartilage, which can become drier, rougher and more fragmented. Iron deposits in the synovial membrane may also trigger the release of enzymes into the joint fluid which can result in damage to the cartilage.
 

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This page was last updated at 12:24PM on August 2, 2021.