Severity of Hemophilia

The severity describes how serious the problem is. The level of severity depends on the amount of clotting factor that is missing from a person’s blood.

NORMAL = 50% - 150%
of the normal activity of clotting factor VIII (8) or IX (9) in the blood.

SEVERE HEMOPHILIA less than 1% of normal clotting factor activity

Bleed often into the muscles or joints (mainly knees, elbows and ankles). Might bleed one or two times per week. Might bleed for no clear reason.

MODERATE HEMOPHILIA 1% - 5% of normal clotting factor activity

Might bleed for a long time after surgery, a bad injury, or dental work. Might bleed about once a month. Rarely bleed for no clear reason.

MILD HEMOPHILIA 5% - 30% of normal clotting factor activity

Might bleed for a long time after surgery or a very bad injury. Might never have a bleeding problem. Do not bleed often. Do not bleed unless injured.

Level Percentage of normal factor activity' in blood Number of international units (IU) per millilitre (ml) of whole blood Normal Range 50%-150% 0.50–1.5 IU Mild Hemophilia 5%-30% 0.05–0.40 IU Moderate Hemophilia 1%-5% 0.01¬–0.05 IU Severe Hemophilia less than 1% less than 0.01 IU

Types of Hemophilia

Acquired Hemophilia

In rare cases, a person can develop hemophilia later in life. The majority of cases involve middle-aged or elderly people, or young women who have recently given birth or are in the later stages of pregnancy. Acquired hemophilia is usually caused by the development of antibodies to factor VIII (the body’s immune system destroys its own naturally produced factor VIII). This condition often resolves with appropriate treatment, which typically involves a combination of steroid treatment and the drug Cyclophosphamide.

There are Two Types of Hemophilia

• Hemophilia A (Classical hemophilia) - Hemophilia A is caused by deficiency of factor VIII
• Hemophilia B (Christmas disease) - Hemophilia B is caused by deficiency of factor IX

People with severe Hemophilia usually bleed frequently into their muscles or joints. They may bleed one or two times per week. Bleeding is often spontaenous, which means the bleeding just happened with no obvious cause.

People with moderate hemophilia bleed less frequently, usually after an injury, perhaps once a month. Cases of hemophilia vary, however, and a person with moderate hemophilia can bleed spontaneously.

People with mild hemophilia usually bleed only as a result of surgery or major injury. They may never have a bleeding problem.