The condition known as von Willebrand disease or vWD is a mild to moderate hemorrhagic disorder caused most commonly by a mutation in a coagulation factor known as the von Willebrand factor (vWF).
This may manifest either in childhood or in later life, with symptoms varying according to the time of life. In general, type 1 has mild symptoms, while type 3 shows the most severe. However, symptom severity depends mostly on the factor VIII levels.
In most types of vWD, mucocutaneous bleeding of varying severity is a prominent manifestation, in the form of epistaxis, gingival bleeding, and easy bruising. Such bleeding is almost always induced by trauma, and spontaneous bleeding is rare, even in severe vWD. Musculoskeletal bleeding, in the form of hemarthroses or hematomas in muscle, occurs only in type 3 vWD, which is quite rare.
Type 1 shows very mild symptoms, but women may complain of heavy menstrual bleeding and postpartum blood loss. In patients with vWF levels lower than 15 IU/dL, symptoms are correspondingly more severe.
Type 2 has several different subtypes, of which types 2A and 2B individuals have mild to moderate bleeding from the skin and mucous membranes. Type 2B patients may, however, develop a low platelet count which drops further during stress-inducing conditions such as pregnancy or stress, or with the use of desmopressin.
Type 2M also has a mild to moderate spectrum of mucocutaneous bleeding manifestations, which may become severe if the levels of vWF ristocetin cofactor are really low or even absent. Type 2N produces symptoms similar to mild hemophilia A because it results in a very low factor VIII level.
Type 3 is associated with the most severe symptoms, especially spontaneous gastrointestinal bleeding, which may be very difficult to control.
Acquired vWD also presents with mild to moderate bleeding from the nose, gums or skin.
Bleeding in vWD
Mucocutaneous bleeding manifestations in vWD include:
- bruising without any history of trauma
- prolonged and repeated episodes of epistaxis
- bleeding from the oral cavity, such as bleeding gums after brushing or flossing
- prolonged bleeding after dental cleaning or tooth extractions
- excessive or prolonged bleeding after surgery or injury
- heavy postpartum bleeding
- In women, menorrhagia dating from menarche is the most common symptom, seen in over 70% of such patients. More than half of such women report dysmenorrhea.
Postpartum hemorrhage is rare in type 1, but so common in other types that replacement therapy is always adopted to prevent it. Similarly, prophylaxis against immediate or delayed postoperative hemorrhage is always required in type 3, but such bleeding is almost never observed in type 1.