Von Willebrand Disease Symptoms and Treatment

Updated on August 22, 2022

Von Willebrand disease is the most common inherited bleeding disorder, affecting approximately one% of the population.

Von Willebrand gene is a blood protein that binds to factor VIII (a coagulation factor). When cistron VIII is not bound to Von Willebrand cistron, information technology breaks down easily. Von Willebrand gene also helps platelets to attach to sites of injury.

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Symptoms

Some patients never experience any significant bleeding.

Von Willebrand disease is often associated with:

  • Like shooting fish in a barrel bruising
  • Prolonged nosebleeds
  • Bleeding from gums
  • Prolonged bleeding afterwards tooth extraction or injury
  • Blood in stool
  • Hematuria (blood in urine)
  • Menorrhagia (excessive menstrual bleeding)
  • Joint bleeding or soft tissue haemorrhage may occur in severe forms like to hemophilia

Types

  • Type one: This is the most common course, occurring in approximately 75% of patients. Information technology is passed down in families in an autosomal dominant fashion meaning only 1 parent needs to be afflicted to laissez passer down the illness. This results from a lower than normal Von Willebrand factor level. Bleeding can be mild to severe.
  • Type 2: Type 2 occurs when the Von Willebrand factor does non part normally.
  • Type 2A: Passed down in autosomal dominant fashion. Moderate to moderately severe haemorrhage.
  • Type 2B: Passed down in autosomal dominant fashion. Moderate to moderately severe bleeding. Thrombocytopenia (depression platelet count) is not uncommon.
  • Type 2M: Uncommon type passed downward in autosomal dominant style.
  • Blazon 2N: Uncommon type passed down in autosomal recessive fashion. This ways the affected patient receives two copies of the mutated gene, one from each parent. Factor 8 levels tin can exist extremely low. Bleeding can be severe and may be confused with hemophilia A.
  • Type 3: This is a rare type of Von Willebrand disease. It is passed down in autosomal ascendant fashion. Bleeding tin can exist severe. Patients with this type accept an extremely low amount or absent-minded Von Willebrand gene. This, in plow, causes a deficiency in cistron 8 and significant haemorrhage.
  • Caused: This form of Von Willebrand disease is caused by something else similar cancer, autoimmune disorders, cardiac anomalies (like ventricular septal defect, aortic stenosis), medications or hypothyroidism.

Diagnosis

First, your dr. must be suspicious that you have a haemorrhage disorder based on the symptoms higher up. Having other family unit members with like symptoms increases suspicion for Von Willebrand disease, particularly if both males and females are affected (in contrast to hemophilia that predominantly bear on males).

Von Willebrand disease is diagnosed past performing a panel of blood work that looks at both the amount of Von Willebrand factor in the claret equally well as its function (ristocetin cofactor activeness). Because several types of Von Willebrand illness can cause a reduction in factor VIII, levels of this clotting protein are also sent. Von Willebrand multimers, which looks at the structure of the Von Willebrand factor and how it is cleaved down, is important particularly in diagnosing Type ii disease.

Treatments

Mildly affected patients may never require treatment.

  • DDAVP: DDAVP (also called desmopressin) is a synthetic hormone that is administered via a nasal spray (or occasionally through an Four). This hormone helps the torso to release Von Willebrand factor stored in the blood vessels.
  • Von Willebrand factor replacement: Like to replacement factor used in hemophilia, infusions of Von Willebrand factor can exist given to foreclose or treat haemorrhage. These products also contain factor Viii too.
  • Antifibrinolytics: These medications (brand names Amicar and Lysteda), typically given orally, help to stabilize clot formation. These can be peculiarly useful for nosebleeds, mouth bleeding, and menstrual haemorrhage.
  • Contraceptives: In women with Von Willebrand disease and heavy menstrual bleeding, hormonal contraceptives like birth control pills or intrauterine devices may be used to reduce/cease haemorrhage.

Past Amber Yates, Physician
Amber Yates, MD, is a board-certified pediatric hematologist and a practicing physician at Baylor Higher of Medicine.

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