血管性血友病与血友病：病因、症状、诊断和治疗的比较

Von Willebrand disease (VWD) and 血友病 are inherited blood disorders. Both disorders make it harder for your blood to clot. While some symptoms may overlap, these conditions aren’t the same. Differences also exist in the way they are diagnosed and treated. 

Von Willebrand Disease vs. Hemophilia: A Quick Overview

Von Willebrand disease (VWD) is the most common inherited bleeding disorder in which the blood doesn’t clot normally. Over 3 million people in the United States live with VWD [1].

VWD happens when the blood is deficient in a clotting protein (clotting factor) called von Willebrand factor. It can also result due to a defective von Willebrand factor. 

Hemophilia is also an inherited bleeding disorder in which the affected individual lacks clotting factor VIII or IX, or rarely XI. It is less common than VWD, affecting about 30,000 Americans [2].

Hemophilia primarily affects males, while VWD is as common in males as it is in females.

VWD symptoms tend to be milder than those with hemophilia.

Von Willebrand Disease vs. Hemophilia: Comparing the Symptoms

VWD and hemophilia share several symptoms, including:

• Easy bruising 
• Recurring or severe nosebleeds
• Unusual bleeding after a trauma or injury
• Heavy bleeding after minor surgery, dental work, or vaccination
• Heavy or abnormally long menstrual bleeding

Internal bleeding may occur in severe cases, leading to:

• Painful or stiff joints and muscles 
• Accumulation of blood under the skin (hematoma)
• 尿液或粪便中带血
• Low number of red blood cells (anemia)

People with VWD often report bleeding in the nose, mouth, intestines, uterus, and vagina. On the other hand, hemophilia is more likely to cause bleeding in the joints and within muscles.

What Test Is Used To Differentiate von Willebrand Disease from Hemophilia?

Blood Tests for VWD 

• Von Willebrand factor antigen

This test measures blood levels of von Willebrand factor.

• Von Willebrand factor activity

This test helps determine if the von Willebrand factor works properly during the clotting process. 

• Factor VIII clotting activity

This measures the level and activity of factor VIII.

• Von Willebrand factor multimer

This helps your healthcare provider determine the type of von Willebrand disease. 

Diagnostic Tests for Hemophilia

• Screening tests

These include blood tests that measure how long your blood takes to form a clot. 

• Factor assays

Also called clotting factor tests, they measure how well clotting factors VIII and IX work during the clotting process. 

Von Willebrand Disease vs. Hemophilia: Comparing Treatments

Von Willebrand Disease Treatment

Minor bleeds in von Willebrand disease might not require treatment. In such cases, your healthcare provider will ask you to avoid certain medications, such as blood thinners (like aspirin).

Treatment for VWD can vary depending on the type and severity of the symptoms. For milder cases, a medicine called desmopressin acetate is administered, either by injection or a nasal spray. 

In more severe cases, you may need injections of lab-created von Willebrand factors. A healthcare provider may consider such injections in milder cases that don’t improve with the desmopressin acetate nasal spray. 

血友病治疗

Hemophilia treatment involves injections of lab-created clotting factors to replace missing factor VIII or IX, depending on the type of hemophilia. Treatment products may include:

• Emicizumab (Hemlibra)
• Desmopressin acetate
• Amicar
• Gene therapy, such as Roctavian and Hemgenix

Von Willebrand Disease vs. Hemophilia: Frequently Asked Questions

Is factor VIII the same as von Willebrand?

No. Though they work closely as a complex (FVIII/VWF), they are different proteins. 

Why is it called von Willebrand disease?

The disease is named after Finnish physician Erik Adolf von Willebrand. In 1926, he published a paper describing bleeding disorders in three generations. He also distinguished the condition from hemophilia and other bleeding disorders known at the time. 

What are other names for the von Willebrand factor?

Other names for the von Willebrand factor are factor VIII-related antigen and ristocetin cofactor. However, health experts rarely use these names nowadays.