Types of VWD

Low von Willebrands Factor (Low vWF)

This is the most common condition, affecting 60% of people identified with reduced vWF levels (Fig 2). Recent research in Ireland, 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

Type 1

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.

Type 2

People with type 2 vWD will make vWF but the quality of the vWF produced is not as good as normal vWF, so it 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.

Type 3

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 you have. Many people have some of the following symptoms and may not know they have vWD.

Common symptoms include:

  • 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.
  • Less common symptoms:
  • Joint or muscle bleeding
  • Gastrointestinal bleeding (bleeding in stomach or gut)

For women:

  • Heavy or prolonged menstrual bleeding
  • Heavy bleeding after surgery or childbirth.

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, you might not even notice, and it may 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 liked continued heavy or prolonged nosebleeds, some dental procedure, 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

For women

  • If you are pregnant
  • If you are having a hysterectomy