Table of Contents
What is Type 2B von Willebrand disease?
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.
Does von Willebrand disease cause low platelet count?
Von Willebrand disease (VWD) is a hereditary quantitative deficiency or functional abnormality of von Willebrand factor (VWF), which causes platelet dysfunction. Bleeding tendency is usually mild. Screening tests usually show a normal platelet count and, possibly, a slightly prolonged partial thromboplastin time (PTT).
What is von Willebrand type 2a?
Disease definition. A subtype of type 2 von Willebrand disease characterized by a bleeding disorder associated with a decrease in the affinity of the Willebrand factor (VWF) for platelets and the subendothelium caused by a deficiency of high molecular weight VWF multimers.
Does von Willebrand disease affect immune system?
These 3 types are all inherited. There’s also a rare type that is not inherited called “acquired von Willebrand disease”. This can start at any age and is usually associated with other conditions that affect the blood, immune system or heart.
Should I take Covid vaccine with low platelet count?
A current diagnosis or history of immune thrombocytopenia (ITP) is not a contraindication to receiving the COVID-19 vaccine, as long as your platelet count is above 20,000 (see below). Patients with ITP are encouraged to get a COVID-19 vaccine.
How quickly does DDAVP work?
After the tablet or nasal spray/solution is given, desmopressin usually starts to work within 1 hour. After an injection is given, desmopressin usually starts to work within 15 to 30 minutes.
What are the laboratory features of type 2B von Willebrand disease (vWD)?
Other laboratory features, such as loss of high-molecular-weight VWF multimers and thrombocytopenia, are common in type 2B VWD, but are not universal. 2 The mainstay of type 2B VWD treatment is VWF replacement therapy.
What is the treatment of choice for von Willebrand factor deficiency (vWD)?
Desmopressin (DDAVP) is the treatment of choice for type 1 vWD as it increases endogenous release of FVIII and von Willebrand factor (vWF) and is also used in some subtypes of type 2 vWD. In those patients in whom DDAVP is ineffective or contraindicated, levels can be restored by infusing vWF:FVIII concentrates.
What is the role of 2B vWD in the treatment of vWD?
The mainstay of 2B VWD treatment remains VWF replacement and consideration of other VWD adjunct therapies, such as antifibrinolytics and hormonal therapies for uterine bleeding.
When is secondary long-term prophylaxis indicated for von Willebrand disease (vWD)?
Some patients with VWD, especially type 3 with FVIII:C levels <5 IU/dL, may have frequent hemarthroses or recurrent spontaneous bleeding (eg, epistaxis in infancy) that can benefit from secondary long-term prophylaxis.