Symptoms of von Willebrand disease

Von Willebrand disease is a common hereditary bleeding disease in clinical practice. Patients are prone to bleeding in the skin and mucous membranes, especially nosebleeds and gingival bleeding. Of course, care should be taken to differentiate it from bleeding diseases and not blindly.

1. Von Willebrand disease is a common hereditary bleeding disease in clinical practice. Its pathogenesis is the patient's von Willebrand factor (vWF) gene mutation, which leads to a decrease in the amount or abnormal quality of plasma vWF. Foreign reports show that the incidence of vWD is about one in a thousand. There is no statistical data in our country yet. Due to the different types of vWD, the clinical bleeding manifestations vary greatly, laboratory tests are more complicated than hemophilia, and the changes in mild patients are atypical, often requiring a combination of medical history and clinical comprehensive judgment.

2. Clinical manifestations

vWD is mostly inherited as an autosomal dominant trait, and a few are inherited as an autosomal recessive trait. Both men and women can be affected.

Patients have a tendency to have skin and mucous membrane bleeding, with epistaxis and gingival bleeding being the most common. This is very different from the clinical manifestations of hemophilia, which are mainly joint and soft tissue bleeding. Female patients often experience heavy menstrual bleeding or postpartum bleeding. The severity of clinical symptoms varies depending on the type of vWD. In mild cases (type 1), the only symptoms are menorrhagia, which may not stop after tooth extraction or other minor surgery, or may be discovered during family investigations; in type 2, the bleeding risk is moderate; in severe cases (type 3), patients have obvious bleeding and may also have spontaneous joint and muscle bleeding like hemophilia. The manifestations and severity of bleeding vary among family members. The patient's bleeding tendency may decrease with age.

3. Differential Diagnosis

1. Differentiate from all bleeding disorders.

2. Bleeding induced by abnormal liver function: The best way to distinguish bleeding related to vitamin K deficiency from bleeding related to liver failure is to measure the content of coagulation factor V. Factor V is synthesized by the liver and is independent of vitamin K. In patients with severe liver disease, factor V and vitamin K-dependent clotting factors are all reduced; in patients with vitamin K deficiency, factor V levels are normal.

3. Differential diagnosis of other diseases: