Von Willebrand-Jürgens syndrome: symptoms, diagnosis, therapy

Von Willebrand-Jürgens syndrome can cause longer and heavier bleeding. How can a blood clotting disorder be recognized?

Von Willebrand-Jürgens syndrome (also called Von Willebrand syndrome) is usually congenital, but is sometimes only diagnosed late. An increased tendency to bleed occurs when a certain protein – the so-called von Willebrand factor – is too little or not present at all or has defects.

Von Willebrand factor plays an important role in blood clotting. If it is not present in sufficient quantities in the body or is defective, it makes blood clotting more difficult. As a result, it takes longer for a wound to stop bleeding. In some cases, bleeding can also become more severe.

The symptoms that occur in individual cases with Von Willebrand-Jürgens syndrome can vary greatly. The extent of the symptoms depends primarily on how severe the von Willebrand factor deficiency or how defective the protein is.

In most cases, Von Willebrand-Jürgens syndrome is only noticeable through mild symptoms – in some cases there are no symptoms at all. Von Willebrand-Jürgens syndrome is therefore not always easy to recognize as such.

An increased bleeding tendency can be a sign of von Willebrand-Jürgens syndrome, even if it is only mild. The possible symptoms that may then occur include:

• frequent bruises (bruises), even with barely noticeable impacts, often not on the arms or legs, but in rather unusual places
• Frequently petechiae, i.e. small hemorrhages in the skin that are no larger than the size of a pinhead
• frequent and prolonged nosebleeds, often seemingly for no reason
• Heavy or prolonged bleeding after minor injuries (e.g. after a cut)
• Heavy or prolonged bleeding after dental procedures (such as tooth extraction or minor procedures in the mouth)
• Bleeding or bruising after blood tests or after an injection or vaccination into the muscle
• in children: sometimes heavy or prolonged bleeding when changing teeth
• frequent bleeding gums after brushing your teeth
• Severe or prolonged bleeding after surgical procedures or accidents with major injuries
• Traces of blood in the stool
• Blood in urine

In girls and women, Von Willebrand-Jürgens syndrome can also be manifested by heavier and longer periods. Possible signs that heavy menstrual bleeding is caused by Von Willebrand-Jürgens syndrome include:

Tampons or pads must be changed often (more often than once an hour) Those affected must use tampons and pads combined. Larger blood clots (diameter over 2.5 centimeters) can be found in the menstrual blood.

Due to the increased blood loss during menstruation, affected women may also experience signs of anemia, which is manifested by tiredness, exhaustion, shortness of breath and paleness.

Von Willebrand-Jürgens syndrome is rare, but is the most common congenital bleeding disorder. It affects an estimated 6 to 10 people in 1,000. However, significantly fewer of these require treatment, namely only around one in 10,000 people.

Experts also differentiate between three types of Von Willebrand-Jürgens syndrome. The most common, type 1, is usually mild. Type 3 of the disease is the rarest – it is estimated to occur in one in a million people.

Since Von Willebrand-Jürgens syndrome is often only mild, it is sometimes diagnosed late or even never. The first indications are usually symptoms such as frequent bruises, frequent nosebleeds or an increased tendency to bleed. If the disease is also present in other family members, such as the mother or father or siblings, von Willebrand-Jürgens syndrome is suspected.

Von Willebrand-Jürgens syndrome can be detected using blood tests. The key factors here are the concentration of von Willebrand factor and factor VIII. The bleeding time and blood values ​​such as the activated partial thromboplastin time (aPTT), the prothrombin time (PT-INR) or the platelet count are also of interest.

To be sure of the diagnosis, it is advisable to repeat the blood test every two weeks. Various factors such as stress or physical exertion can influence blood values.

Since Von Willebrand-Jürgens syndrome is mild in most cases, it often does not require any further treatment. Even in severe cases, people with Von Willebrand-Jürgens syndrome often only need treatment in certain situations, rather than on a daily basis. Therapy is usually limited to avoiding medications that inhibit blood clotting. This particularly applies to the active ingredient acetylsalicylic acid (ASS).

In order to be able to intervene appropriately in the event of bleeding in von Willebrand-Jürgens syndrome, it is fundamentally important for those affected and for doctors to know exactly which type of blood clotting disorder is present.

In people with Von Willebrand-Jürgens syndrome, light bleeding can often be stopped without additional medication. To stop the bleeding, it is often enough to apply some pressure to the wound for a few minutes. If you have a nosebleed, squeezing your nostrils together with your fingers can help.