About Pseudo-von Willebrand disease

What is Pseudo-von Willebrand disease?

Pseudo-von Willebrand disease (PvWD) is a rare inherited bleeding disorder caused by a defect in the von Willebrand factor (VWF) gene. It is characterized by a mild to moderate bleeding tendency, similar to that seen in von Willebrand disease (VWD). However, unlike VWD, the VWF levels in the blood of those with PvWD are normal or only slightly reduced.

What are the symptoms of Pseudo-von Willebrand disease?

The symptoms of Pseudo-von Willebrand disease (PvWD) vary from person to person, but may include:

- Prolonged bleeding after minor cuts, surgery, or dental procedures

- Easy bruising
- Excessive bleeding during menstrual periods
- Nosebleeds
- Blood in the urine or stool
- Heavy bleeding after childbirth
- Excessive bleeding after a tonsillectomy or other surgery
- Excessive bleeding after a tooth extraction
- Excessive bleeding after a circumcision
- Excessive bleeding after a biopsy

What are the causes of Pseudo-von Willebrand disease?

Pseudo-von Willebrand disease (PvWD) is caused by a genetic mutation in the VWF gene, which is responsible for producing von Willebrand factor (VWF). This mutation results in a decrease in the amount of VWF produced, leading to a decrease in the ability of the blood to clot. Other causes of PvWD include autoimmune disorders, certain medications, and certain types of cancer.

What are the treatments for Pseudo-von Willebrand disease?

The treatment for Pseudo-von Willebrand disease (PvWD) depends on the severity of the condition and the symptoms experienced by the patient. Treatment options may include:

1. Desmopressin (DDAVP): This is a synthetic hormone that helps to increase the levels of von Willebrand factor in the blood.

2. Platelet transfusions: Platelets are blood cells that help with clotting. A transfusion of platelets can help to increase the levels of von Willebrand factor in the blood.

3. Antifibrinolytic drugs: These drugs help to reduce the breakdown of clotting factors in the blood.

4. Surgery: In some cases, surgery may be necessary to repair damaged blood vessels or to remove a tumor that is causing the condition.

5. Blood

What are the risk factors for Pseudo-von Willebrand disease?

1. Family history of von Willebrand disease
2. Female gender
3. Certain medications, such as aspirin, ibuprofen, and other non-steroidal anti-inflammatory drugs
4. Certain medical conditions, such as thyroid disease, diabetes, and liver disease
5. Certain genetic mutations, such as those in the VWF gene
6. Certain autoimmune disorders, such as lupus and rheumatoid arthritis
7. Certain infections, such as HIV and hepatitis C

Is there a cure/medications for Pseudo-von Willebrand disease?

There is no cure for Pseudo-von Willebrand disease, but medications can be used to help manage the symptoms. These medications include desmopressin (DDAVP), antifibrinolytic agents, and antifibrinolytic agents combined with desmopressin. Additionally, platelet transfusions may be used to help manage bleeding episodes.