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Acquired hemophilia B is extremely rare most cases of acquired hemophilia are in those with hemophilia A or factor VIII deficiency. The factor IX antibodies destroy circulating factor IX in the blood causing bleeding symptoms. Acquired hemophilia B is caused by the body’s production of antibodies against its own factor IX protein. An individual with acquired hemophilia B is not born with the condition. Through her descendants, the disorder was passed down to the royal families of Germany, Spain and Russia and thus hemophilia B is also known as the “royal disease.”Īlthough the focus of this report is the genetic, or inherited, form of hemophilia B, it should be noted that another form called acquired hemophilia B can develop, most commonly later in life (see “Related Disorders” section below). The most well-known family with hemophilia B was that of Queen Victoria of England. The disorder was first reported in the medical literature in 1952 in a patient with the name of Stephen Christmas. Hemophilia B, also known as factor IX deficiency or Christmas disease, is the second most common type of hemophilia. Hemophilia B is most commonly identified in males however, females who carry the gene may have mild or, rarely, more severe symptoms of bleeding and should have their factor IX level checked. Hemophilia B is caused by changes (variants or mutations) in the factor IX gene ( F9) on the X chromosome. In some moderate and most severe cases, bleeding symptoms may occur after a minor injury or spontaneously, meaning without an identifiable cause. In mild cases, bleeding symptoms may occur only after surgery, injury or a dental procedure. Hemophilia B is classified as mild, moderate or severe based upon the activity level of factor IX. This is sometimes referred to as prolonged bleeding or a bleeding episode. This is because they are missing or have a decreased amount of a protein involved in blood clotting and are unable to effectively stop the flow of blood from a wound, injury or bleeding site. Individuals with hemophilia B do not bleed faster than unaffected individuals, they bleed longer. Clotting factors are specialized proteins needed for blood clotting, the process by which blood seals a wound to stop bleeding and promote healing.
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Hemophilia B is a rare genetic bleeding disorder in which affected individuals have insufficient levels of a blood protein called factor IX. Stay Informed With NORD’s Email Newsletter.Find a Rare Disease Patient Organization.Rare Disease Cures Accelerator (RDCA-DAP).Find Clinical Trials & Research Studies.Launching Registries & Natural History Studies.A Podcast For The Rare Disease Community.