Hemophilia is a rare genetic blood clotting disorder that primarily affects males. People with hemophilia do not produce enough of, or are missing, certain blood clotting proteins called factors. The body depends on these clotting factors to stop bleeding after injury and to promote healing. When a person with hemophilia gets a cut or bleeds internally, it results in longer bleeding episodes. Internal bleeding is usually into joints and muscles, but can also occur in the brain or other organs.
Hemophilia A or Classic hemophilia, the clotting factor VIII is either absent or not present in sufficient amounts, and Hemophilia B or Christmas disease, the clotting factor IX is either absent or not present in sufficient amounts. Virtually all people who have hemophilia A or B are born with it and the majority of people with hemophilia have a family history. However, in as many as 30% of cases, there is no family history of hemophilia. In these cases, the mother may not be aware that she carries the gene for hemophilia, or a gene mutation may have occurred spontaneously. Treatment is based on providing the missing/deficient factor in the form of antihemophilic clotting factor.
Mild hemophilia A patients have factor VIII levels of 6% to 50% of normal.
Moderate hemophilia A patients have factor VIII levels of 1% to 5% of normal.
Severe hemophilia A patients have factor VIII levels of <1% of normal.
There is presently no cure for hemophilia, but with treatment using clotting factors, people with even severe hemophilia can get bleeding under control and enjoy a full and active life.
Print
this page 