How do you treat type 2B von Willebrand disease?

How do you treat type 2B von Willebrand disease?

As with other subtypes of vWD, the mainstay of therapy for type 2B vWD is vWF replacement therapy, in the form of recombinant vWF (rVWF) or vWF/FVIII concentrates. The adjunct therapies useful in vWD also apply in this variant. These include: antifibrinolytics (aminocaproic acid, tranexamic acid)

What is the best treatment for VWD?

Desmopressin. This medication is available as an injection (DDAVP). It’s a synthetic hormone that controls bleeding by stimulating your body to release more of the von Willebrand factor stored in the lining of your blood vessels. Many doctors consider DDAVP the first treatment for managing von Willebrand disease.

What is von Willebrand type 2B?

Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder caused by changes in von Willebrand factor (VWF) that enhance binding of VWF to GPIb on platelets.

What medications should be avoided with von Willebrand disease?

Avoid nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Advil or Motrin, for example), and naproxen (Aleve). Take medicine to prevent heavy bleeding if you have an injury, are going to have surgery, or are about to give birth. Avoid medicines (called blood thinners) that prevent blood clots.

What is platelet aggregation?

Platelet aggregation, the process by which platelets adhere to each other at sites of vascular injury, has long been recognized as critical for hemostatic plug formation and thrombosis.

What is platelet type VWD?

Platelet-type von Willebrand disease (PT-VWD) is a rare autosomal dominant bleeding disorder which is due to a mutation in the gene encoding for platelet glycoprotein Ibα (GPIbα) resulting in enhanced affinity for von Willebrand factor (VWF).

How does desmopressin increased VWF?

Desmopressin exerts its haemostatic effect by: Inducing synthesis of the von Willebrand factor (VWF) by endothelial cells. Stimulating release of the VWF from its storage sites in endothelial cells. Cleaving the large VWF multimers circulating in plasma into smaller multimers.

How do you treat type 2 variants of von Willebrand disease?

Replacement therapy with plasma-derived von Willebrand factor-factor VIII concentrates represents the safe mainstay of treatment of all patients, particularly those not responding to desmopressin or requiring a sustained hemostatic correction because of major surgery or bleeding.

What foods should be avoided with von Willebrand disease?

Food and supplements to avoid

• large glasses of juice.
• soft drinks, energy drinks, and sweetened tea.
• heavy gravies and sauces.
• butter, shortening, or lard.
• full-fat dairy products.
• candy.
• foods containing trans fats, including fried. foods and baked goods (pastries, pizza, pie, cookies, and crackers)

How can VWD be prevented?

Because it’s a genetic condition someone is born with, there is no way to prevent the disease, so scientists are constantly investigating ways that the disease can be stopped before it passes to the next generation.

What is the best test for platelet function?

Platelets can be evaluated for functional defects using flow cytometry. This test uses lasers to determine proteins that are present on the platelet surface and how they change when the platelet is activated.

Which is the best treatment for type 2B vWD?

The mainstays of type 2B vWD treatment remain vWF replacement and vWD adjunct therapies (including antifibrinolytics and topical hemostatic agents). Treatment with DDAVP is controversial but may be a convenient option for some patients with minor bleeding.

How is type 2B von Willebrand disease treated?

Treatment is similar to other types of vWD, but special consideration should be given to situations that can worsen thrombocytopenia, such as pregnancy. The mainstays of type 2B vWD treatment remain vWF replacement and vWD adjunct therapies (including antifibrinolytics and topical hemostatic agents).

Which is the second most common type of vWD?

This is a common and complex inherited bleeding disorder, with more than 20 distinct subtypes. Type 2 vWD is the second-most common type of vWD (after type 1 vWD), representing about 15 to 30 percent of all cases.

What causes lack of HMWM in type 2A vWD?

In type 2A VWD, there is a variable lack of HMWM, caused mainly by mutations in the A2 domain affecting the multimerization process or susceptibility to ADAMTS-13, but also in D1/D2 multimerization regions and the C-terminal CK region.