There are many different types of bleeding disorders, with von Willebrand disease (VWD) being the most common. The best-known forms of hemophilia — another type of bleeding disorder — are hemophilia A and B. But there are also rarer types of hemophilia.
Bleeding disorders are classified by which clotting factor is ineffective or lacking. Clotting factors are proteins crucial for blood clotting.
In some cases, hemophilia types are also categorized by their underlying cause. Most people inherit the gene changes that lead to hemophilia from their parents. Acquired hemophilia, however, may develop during your lifetime.
It’s important to know which type of bleeding disorder a person has to determine the most effective treatment. Here’s what you need to know about different types of hemophilia and other bleeding disorders.
Most bleeding disorders are genetic conditions, meaning they’re inherited and passed down through families. Others are acquired, meaning they develop later in life due to causes like certain medications, autoimmune conditions, or other underlying health issues.
For people living with VWD, a protein called von Willebrand factor (VWF) is absent, is present at low levels, or doesn’t work properly. Any of these situations can lead to reduced levels of clotting factor VIII (8). (Clotting factors are usually described with Roman numerals.)
Around 1 in every 100 people in the United States has a form of VWD. There are three subtypes of von Willebrand disease that describe its severity:
In hemophilia A, also known as classic hemophilia, people lack or have low levels of factor VIII. According to the National Bleeding Disorders Foundation, about 1 in 5,617 males in the United States are born with hemophilia.
About 60 percent of people with hemophilia A have the most severe form of the disease. They have very little to no factor VIII and experience frequent spontaneous bleeding episodes without treatment.
People with hemophilia B — also called Christmas disease — lack factor IX. About 5 in 100,000 males worldwide are born with hemophilia B.
A rare form called hemophilia B Leyden affects about 3 percent of people with hemophilia B. This form causes frequent bleeding episodes during childhood, which often subside after puberty.
By adulthood, some people with hemophilia B Leyden may reach near-normal factor IX levels and no longer need treatment for this bleeding disorder.
Some people aren’t born with hemophilia but develop it later in life when their antibodies start to attack clotting factor VIII. Known as acquired hemophilia, this autoimmune condition can be severe.
The cause is typically another health condition or certain medications, but about half of people have idiopathic acquired hemophilia. This means the cause is unknown.
Acquired hemophilia affects up to 1.78 per 1 million people per year in the United States. The condition can improve with treatments such as immunosuppressive drugs to stop the body from attacking itself.
Factor I deficiency, also known as congenital fibrinogen deficiency, affects the body’s production or function of fibrinogen. This is a protein crucial for blood clotting.
Congenital afibrinogenemia is a severe, but rare, form that affects about 1 in 1 million people worldwide.
Factor II deficiency, also known as prothrombin deficiency, affects an estimated 1 in every 2 million people in the general population. The condition may be either inherited or acquired due to factors such as liver disease, vitamin K deficiency, or long-term use of antibiotics.
Factor II deficiency is usually mild, though more severe symptoms can occur.
Factor V deficiency affects about 1 out of every 1 million people in the world. People who have the most severe form of this deficiency typically start experiencing symptoms in childhood. Symptoms may include frequent nosebleeds, easy bruising, and prolonged bleeding after injuries or surgeries.
Factor VII deficiency affects about 1 in 300,000 to 500,000 people in the general world population. The severity of the condition can vary. Some people experience mild to severe symptoms, while others may have none.
Factor VII deficiency can be inherited or, very rarely, acquired due to severe liver disease, vitamin K deficiency, or the use of blood-thinning medications.
Approximately 1 out of every 500,000 to 1 million people worldwide have factor X deficiency, which can range from moderate to severe. Symptoms include prolonged bleeding after injuries, easy bruising, and excessive bleeding.
Factor X deficiency is also known as Stuart-Prower deficiency.
Factor XI deficiency, also known as hemophilia C, affects about 1 in 100,000 people in the United States. This condition typically ranges from mild to moderate in severity. People with factor XI deficiency often experience prolonged bleeding after surgeries or injuries.
Factor XIII deficiency is extremely rare, affecting about 1 out of every 2 million to 5 million people globally. The condition can range from mild to severe. Some researchers believe this condition may be underdiagnosed, as its symptoms can be subtle or mistaken for other bleeding disorders.
In rare cases, some people have deficiencies of more than one clotting factor. The most common combination deficiency involves factors V and VIII. In this condition, the body produces both factors but can’t properly transport them into the bloodstream.
In very rare cases, some babies are born lacking some of the clotting factors that depend on vitamin K — including factors II, VII, IX, and X. This condition, known as vitamin K-dependent clotting factor deficiency, is an inherited disorder that can cause symptoms at birth or later in life.
Another problem with vitamin K, known as vitamin K deficiency bleeding, is common. The early and classical form occurs in about 1 in 60 to 1 in 250 newborns in the United States. It’s preventable with a vitamin K injection at birth.
For hemophilia A and hemophilia B, doctors categorize how severe a person’s bleeding disorder is by testing the level of the clotting factor in their blood. Lower levels of the clotting factor typically mean more severe symptoms.
The type of bleeding disorder you have is central to the treatment plan your healthcare team develops. Treatments may include clotting factor replacement therapies, gene therapy, and non-clotting factor medications that help prevent and manage bleeding episodes.
When discussing treatments, ask your bleeding disorder specialist all of your questions. Tell them about how your condition is progressing, any new symptoms or side effects, and whether your current plan is effective. Partner with your doctor to find a treatment plan you can stick with and that meets your needs.
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Hemophilia. Please tell me about Factor 11.
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