The condition known as von Willebrand disease (vWD) is a mild to a moderate hemorrhagic disorder that is most commonly caused by a mutation in a coagulation factor known as the von Willebrand factor (vWF).
vWD may manifest either in childhood or later in life, with symptoms varying according to the specific type of disease. In general, type 1 is associated with mild symptoms, whereas type 3 often causes patients to experience the most severe symptoms of the disease. Despite these differentiations between the types of vWD and their associated symptoms, symptom severity most often depends on 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, with spontaneous bleeding as a rare occurrence, even in severe vWD patients. Musculoskeletal bleeding in the form of hemarthroses or hematomas in muscle occurs only in type 3 vWD, which is quite rare.
Type 1 vWD
Type 1 vWD is associated with very mild symptoms; however, female vWD patients may complain of heavy menstrual bleeding and postpartum blood loss. In vWD patients with vWF levels lower than 15 IU/dL, symptoms are correspondingly more severe.
Type 2 vWD
Type 2 vWD has several different subtypes, which include types 2A, 2B, 2M, and 2N. Both Type 2A and 2B vWD patients have mild to moderate bleeding from the skin and mucous membranes. Type 2B patients may, however, develop a low platelet count that drops further during stress-inducing conditions such as pregnancy or stress or with the use of desmopressin.
Type 2M is associated with causing a mild to moderate spectrum of mucocutaneous bleeding manifestations, which may become severe if the levels of vWF ristocetin cofactor are particularly low or absent. Type 2N vWD causes symptoms that are similar to mild hemophilia A, as this condition results in very low factor VIII levels.
Type 3 vWD
Type 3 vWD is associated with the most severe symptoms of vWD, the most notable of which include spontaneous gastrointestinal bleeding, which may be very difficult to control.
Acquired vWD
Acquired vWD also presents with mild to moderate bleeding from the nose, gums, or skin.
What is von Willebrand Disease?
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 that is seen in over 70% of vWD female patients. More than half of these women will also report dysmenorrhea.
Postpartum hemorrhage is rare in type 1 vWD; however, this symptom is so common in other types of vWD that replacement therapy is always adopted to prevent its occurrence. Similarly, prophylaxis against immediate or delayed postoperative hemorrhage is always required in type 3 vWD, while such bleeding is almost never observed in type 1 vWD.
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