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Types of Von willebrand Disorder

Low Von Willebrands Factor (Low VWF)

This is the most common condition, affecting 60% of people identified with reduced vWF levels. Recent research in Ireland, carried out on people with Low vWF, identified that bleeding, in particular easy bruising, may be problematic in patients with Low vWF. In the same research, women with heavy periods were also identified as a group who may have problems due to low vWF. As a result, some people with low vWF levels have bleeding symptoms like people with vWD and may need to have treatment if they are having surgery or other invasive procedures. Women with low vWF may also need treatment to help with management of heavy periods.

If you have von Willebrand factor (vWF) levels, less than 0.30 IU/mL(30%), your doctor will do additional tests to figure out what type of von Willebrand Disorder (vWD) you have. This is important as treatment options, responses to treatment and monitoring differ between types of vWD.

VWF Level Diagnosis
50% Normal
30-50% Low VWF Level
< 30% VWD

Types of Von Willebrand Disorder

People who have type 1 vWD have much lower levels of vWF than expected because their body either doesn’t make enough vWF or breaks it down too quickly. Doctors often refer to this as a “quantitative” issue. This means the quantity or the amount of vWF available in the body is not enough. Type 1 is the most common form of those diagnosed with vWD, with about 3 out of 4 people who have VWD, being diagnosed with type 1.

People with type 2 vWD do make vWF but the quality of the vWF is not as good as it is normally. Therefore the vWF doesn’t work well. This can often be referred to as a “qualitative” problem. Type 2 can be further subdivided depending on which part of the vWF is not working correctly (type 2A, 2B, 2M or 2N). Different types of mutations (changes) cause each type. Of those diagnosed with vWD, about 1 in 4 have type 2.

People who have type 3 vWD usually have extremely low levels of vWF. As they do not have enough vWF to carry factor VIII around their body, their factor VIII levels are also low, and they may experience bleeding like people with haemophilia. Type 3 is the most serious form of vWD, but it is rare, affecting about 1 in 500,000 people.

What Are The Symptoms?

The symptoms of vWD and their severity mostly depend on the type of vWD. Many people have some of the following symptoms and may not know they have vWD:

Common Symptoms:
  • Frequent large bruises from minor bumps or injuries.
  • Frequent or prolonged nose bleeds.
  • Prolonged bleeding from gums after dental procedure.
  • Heavy or prolonged bleeding from a cut.
  • Prolonged or heavy bleeding after injury.
  • Heavy or prolonged menstrual bleeding
  • Heavy bleeding after surgery or childbirth.
Less Common Symptoms
  • Joint or muscle bleeding
  • Gastrointestinal bleeding (bleeding in stomach or gut)
Type 3 VWD

People who have type 3 vWD may also have more frequent bleeding into muscles or joints than the other types of vWD. This can occur after minor injuries or over exertion. It may also appear that the bleeding started for no reason.

How Is It Treated?

Bleeding such as bruising, minor cuts and some nosebleeds can often be controlled using simple first aid measures such as R.I.C.E (Rest, Ice, Compression and Elevation). If bleeding continues in cases such as heavy or prolonged nosebleeds, some dental procedures (during or after surgery), or after injury, other treatment options may be needed.

For young women, treatment such as hormonal therapies or the intrauterine devices (IUD’s. e.g. Mirena coil) may be used as bleed prevention for heavy or prolonged bleeding. There are also surgical options if required.

Please contact your Haemophilia Treatment Centre for advice:

  • If you need a dental extraction
  • If you need intramuscular injections
  • If you need surgery
  • If you are injured
  • If you are pregnant
  • If you are having a hysterectomy