Common tests & procedures
von Willenbrand Disease (VWD) Diagnosis. To find out if a person has von Willebrand disease (VWD), the doctor will ask questions about personal and family histories of bleeding. The doctor also will check for unusual bruising or other signs of recent bleeding and order some blood tests that will measure how the blood clots.
How do you test for von Willebrand disease?
Von Willebrand Factor Antigen – This test measures how much VWF is in the blood. It does not tell how well the VWF functions. Ristocetin Cofactor Assay – Normal platelets and the antibiotic ristocetin are added to the tube of plasma from the person being tested.
What is a von Willebrand factor antibody test?
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.
How is von Willebrand disease treated?
von Willenbrand Disease (VWD) Diagnosis 1 Complete Blood Count (CBC). 2 Activated Partial Thromboplastin Time (APTT) Test. 3 Prothrombin Time (PT) Test. 4 Fibrinogen Test.
How to diagnose von willenbrand disease (vWD)?
What is the best test to diagnose von Willebrand's disease?
Diagnostic Tests The blood tests that a doctor can order to diagnose VWD (or another platelet disorder) include: Factor VIII clotting activity―To measure the amount of factor VIII in the blood. Von Willebrand factor antigen―To measure the amount of VWF in the blood.
What is included in a von Willebrand panel?
Von Willebrand factor testing includes VWF antigen, which measures the amount of VWF, and VWF activity (also known as Ristocetin Cofactor), which evaluates the function of VWF. Some laboratories may offer a panel that includes both of these tests along with a factor VIII activity test.
Can you be misdiagnosed with von Willebrand disease?
Despite this, VWD is one of the most commonly misdiagnosed or overlooked entities in everyday clinical practice. Of interest, VWD may be both over- and under-diagnosed, as well as misdiagnosed, either as another entity or as a different subtype of the disorder.
What is a von Willebrand screening test?
A von Willebrand factor (vWF) antigen test measures the amount of a clotting factor called von Willebrand factor. The body's clotting factors work together in a special order, almost like pieces of a puzzle. When the last piece is in place, the clot develops.
When should you suspect von Willebrand disease?
Von Willebrand factor levels of 30 IU per dL or lower are required for the definite diagnosis of inherited von Willebrand disease. Persons with levels of 30 to 50 IU per dL may not have the disease, but may need agents to increase von Willebrand factor levels during invasive procedures or childbirth.
What medications should be avoided with von Willebrand disease?
If you have von Willebrand disease (VWD), you can take steps to prevent bleeding and stay healthy. For example, avoid over-the-counter medicines that can affect blood clotting, such as aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs).
Does von Willebrand disease make you tired?
The weakening, fatigue and other disruptive effects of VWD can be stopped. Even childbirth can be made safe for a mother and baby. If you have unexplained bleeding symptoms, remember to ask your doctor, “Could I have von Willebrand disease?”
Can you have von Willebrand without knowing?
Many people with von Willebrand disease don't know it because the signs are mild or absent. The most common sign of the condition is abnormal bleeding. There are three main types of the disease. The amount of bleeding varies from one person to another, depending on the type and severity of the disease.
What medications can cause von Willebrand disease?
Rarely, acquired von Willebrand disease may also develop in direct association with use of certain medicines....These medicines include:Ciprofloxacin.Griseofulvin.Valproic acid.
Does von Willebrand disease affect platelets?
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).
Can you have surgery with von Willebrand disease?
The results from this retrospective study indicate that surgery can be safely performed by providing adequate and timely hemostasis during and after the procedure in patients with vWD. Perioperative and postoperative bleeding complications are rare when patients are closely and carefully monitored.
What does high von Willebrand activity mean?
VWF is an acute-phase reactant and may be elevated above baseline in association with a variety of conditions including inflammation, stress, exercise, liver disease, pregnancy, or estrogen therapy. Baseline VWF levels also increase with aging.
Can von Willebrand disease have normal PTT?
Von Willebrand Disease (VWD) PTT can be normal or elevated depending on the severity of the disease. Von Willebrand disease is an autosomal dominant deficiency in functional VWF. VWF has 2 functions: binding platelets to the endothelium and protecting Factor VIII. There are 3 types of Von Willebrand disease.
Are platelets low with von Willebrand disease?
Purpose: von Willebrand disease (vWD) is the most common hereditary bleeding disorder and type 2B combines thrombocytopenia. So it must be considered in patients found to have low platelet counts, particularly if there is a family history of mucocutaneous hemorrhage.
Does von Willebrand disease affect platelets?
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).
Does von Willebrand cause low platelets?
Platelet-type von Willebrand disease is an inherited platelet disorder characterized by thrombocytopenia with large platelets caused by gain-of-function variants in GP1BA leading to enhanced GPIbα-von Willebrand factor (vWF) interaction.
How to evaluate for von Willebrand disease?
To evaluate you for von Willebrand disease, your doctor will likely ask you detailed questions about your medical history and check for bruises or other signs of recent bleeding.
Why do von Willebrand tests fluctuate?
The results of these tests can fluctuate in the same person over time due to factors such as stress, exercise, infection, pregnancy and medications. So you might need to repeat some tests. If you have von Willebrand disease, your doctor might suggest ...
What is the first treatment for von Willebrand disease?
Many doctors consider DDAVP the first treatment for managing von Willebrand disease. It can be used before minor surgical procedures to help control bleeding. You might be given a trial of desmopressin to make sure it's effective for you.
What is the Von Willebrand factor?
Von Willebrand factor antigen. This determines the level of von Willebrand factor in your blood by measuring a particular protein. Von Willebrand factor activity. There are a variety of tests to measure how well the von Willebrand factor works in your clotting process. Factor VIII clotting activity.
How long do you have to fast for a blood test?
Ask about pre-appointment restrictions when you make the appointment. You might need to follow dietary restrictions or fast for 8-10 hours if blood tests are scheduled.
Can von Willebrand disease be diagnosed?
Diagnosis. Mild forms of von Willebrand disease can be difficult to diagnose because bleeding is common, and, for most people, doesn't indicate a disease. However, if your doctor suspects you have a bleeding disorder, he or she might refer you to a blood disorders specialist (hematologist). To evaluate you for von Willebrand disease, your doctor ...
When to reschedule a procedure?
If you're having a procedure that's not urgent, reschedule it until after you've been evaluated for a bleeding disorder.
What is the Von Willebrand test?from urmc.rochester.edu
Von Willebrand functional assay. This test measures how well your von Willebrand factor is working.
What is the Von Willebrand factor multimer analysis?from urmc.rochester.edu
Von Willebrand factor multimer analysis. This test finds out if you have one of the subtypes of type 2 von Willebrand disease.
What is this test?from urmc.rochester.edu
This panel of tests is used to diagnose von Willebrand disease. This is a bleeding disorder that causes excessive bleeding from minor injuries or normal physical processes such as menstruation. It's the most common inherited bleeding disorder, but few people with the disease have symptoms.
What other tests might I have along with this test?from urmc.rochester.edu
If your first test results are abnormally low or your healthcare provider needs more information, you may need more specialized blood tests . These may include:
What do my test results mean?from urmc.rochester.edu
Test results may vary depending on your age, gender, health history, the method used for the test, and other things. Your test results may not mean you have a problem. Ask your healthcare provider what your test results mean for you.
How is this test done?from urmc.rochester.edu
The test is done with a blood sample. A needle is used to draw blood from a vein in your arm or hand.
What might affect my test results?from urmc.rochester.edu
Anxiety and stress can make your levels of von Willebrand factor and factor VIII go up. Exercising shortly before the test also can cause a rise in von Willebrand factor.
How long does it take to get von Willebrand test results?
It may take up to one week for your doctor to receive the blood test results. If any of the results suggest a bleeding disorder, the doctor may recommend repeating certain tests and that family members also be tested to see if they have the condition.
How much does von Willebrand test cost?
Results: The cost of testing adolescents with menorrhagia for VWD was $1790, versus $1251 for not testing for VWD. The effectiveness of not testing in quality-adjusted life-years (QALYs) gained (14.237 QALYs) was similar to the VWD testing strategy (14.246 QALYs).
What is in a von Willebrand panel?
Von Willebrand factor testing includes VWF antigen, which measures the amount of VWF, and VWF activity (also known as Ristocetin Cofactor), which evaluates the function of VWF. Some laboratories may offer a panel that includes both of these tests along with a factor VIII activity test.
What is normal range of VWF?
In the general population, the mean level of plasma VWF is 100 IU per dL, with a normal reference range between 50 and 200 IU per dL. The 5 percent of persons with VWF levels of less than 50 IU per dL include those with VWD and those with slightly low, but nondiagnostic, levels.
Can you take ibuprofen with von Willebrand disease?
Treatment depends on the type of von Willebrand’s disease you have, how much you bleed, and your risk for heavy bleeding. You may need to: Avoid nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Advil or Motrin, for example), and naproxen (Aleve).
What is Type 3 von Willebrand disease?
Disease definition. A form of von Willebrand disease (VWD) characterized by a bleeding disorder associated with a total or near-total absence of Willebrand factor (VWF) in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII (FVIII).
Is von Willebrand factor the same as Factor VIII?
Factor VIIII (FVIII) and von Willebrand factor (VWF) are two distinct but related glycoproteins that circulate in plasma as a tightly bound complex (FVIII/VWF).
How long to centrifuge plasma?
Centrifuge for 10 minutes and carefully remove 2 / 3 of the plasma using a plastic transfer pipette, being careful not to disturb the cells. Deliver to a plastic transport tube, cap, and recentrifuge for 10 minutes. Use a second plastic pipette to remove the plasma, staying clear of the platelets at the bottom of the tube.
What is the vwf protein?
vWF is produced by megakaryocytes and endothelial cells. 8 The vWF protein polymerizes in plasma into multimers of up to 100 subunits that range in size from 0.5 to 20 million daltons. 6,8 vWF's plasma half-life is about 24 hours.
Is vwd rare?
Acquired vWD is very rare with less than 100 cases documented in the literature. 13 The majority of cases have been reported in association with autoimmune or clonal proliferative conditions. 13 Acquired vWD has been reported in patients with systemic lupus erythematosus, antiphospholipid syndrome, and hypothyroidism. Other conditions associated with acquired vWD are Wilms' tumor and congenital cardiovascular disease. 8,13 Autoantibodies to vWF can sometimes cause vWD in elderly individuals with multiple myeloma, lymphoproliferative disorders, or collagen vascular disease. 8
What blood test is performed for von Willebrand disease?from en.wikipedia.org
Basic tests performed in any patient with bleeding problems are a complete blood count -CBC (especially platelet counts), activated partial thromboplastin time -APTT, prothrombin time with International Normalized Ratio-PTINR, thrombin time-TT, and fibrinogen level. Patients with abnormal tests typically undergo further testing for hemophilias. Other coagulation factor assays may be performed depending on the results of a coagulation screen. Patients with von Willebrand disease typically display a normal prothrombin time and a variable prolongation of partial thromboplastin time.
How much chance of passing von Willebrand disease to children?from mayoclinic.org
If you have the gene for von Willebrand disease, you have a 50% chance of transmitting this gene to your children. The most severe form of the condition is "autosomal recessive," which means both of your parents have to pass an abnormal gene to you.
How does VWD testing affect the results?from en.wikipedia.org
The testing for VWD can be influenced by laboratory procedures. Numerous variables exist in the testing procedure that may affect the validity of the test results and may result in a missed or erroneous diagnosis. The chance of procedural errors are typically greatest during the preanalytical phase (during collecting storage and transportation of the specimen) especially when the testing is contracted to an outside facility and the specimen is frozen and transported long distances. Diagnostic errors are not uncommon, and the rate of testing proficiency varies amongst laboratories, with error rates ranging from 7 to 22% in some studies to as high as 60% in cases of misclassification of VWD subtype. To increase the probability of a proper diagnosis, testing should be done at a facility with immediate on-site processing in a specialized coagulation laboratory.
What is a DDAVP injection?from cdc.gov
Desmopressin Acetate Injection. This medicine (DDAVP ®) is injected into a vein to treat people with milder forms of VWD (mainly Type 1) . It works by making the body release more VWF into the blood. It also helps increase the level of factor VIII in the blood.
What is VWD in blood?from en.wikipedia.org
Platelet-type VWD (also known as pseudo-VWD) is an autosomal dominant genetic defect of the platelets. The VWF is qualitatively normal and genetic testing of the von Willebrand gene and VWF protein reveals no mutational alteration. The defect lies in the qualitatively altered GPIb receptor on the platelet membrane which increases its affinity to bind to the VWF. Large platelet aggregates and high molecular weight VWF multimers are removed from the circulation resulting in thrombocytopenia and diminished or absent large VWF multimers. The ristocetin cofactor activity and loss of large VWF multimers are similar to VWD type 2B.
What are the complications of von Willebrand disease?from mayoclinic.org
Rarely, von Willebrand disease can cause uncontrollable bleeding, which can be life-threatening. Other complications of von Willebrand disease can include: 1 Anemia. Women who have heavy menstrual bleeding can develop iron deficiency anemia. 2 Swelling and pain. This can be a result of abnormal bleeding in the joints or soft tissue.
How long does it take for blood to ooze after VWD surgery?from cdc.gov
The surgery site oozes blood longer than 3 hours after the surgery. The surgery site needs packing or cautery to stop the bleeding. The amount of bleeding depends on the type and severity of VWD. Other common bleeding events include: Blood in the stool (feces) from bleeding into the stomach or intestines.
What are the symptoms of von Willebrand disease?
Symptoms of von Willebrand disease can include easy bruising, frequent nosebleeds, excessive bleeding after a mouth injury or dental work, abnormal menstrual bleeding, or, in infant boys, prolonged bleeding after circumcision. Von Willebrand disease also may be suspected when a child has a family member with a bleeding disorder.
What is a von Willebrand factor?
What It Is. A von Willebrand factor (vWF) antigen test measures the quantity of a protein called von Willebrand factor that helps blood to clot. A clot is a lump of blood that the body produces to prevent excessive bleeding by sealing leaks in blood vessels caused by wounds, cuts, scratches, and other conditions.
What is the VWF activity test?
These may include: vWF activity – ristocetin cofactor (which measures how well the von Willebrand factor is working) the factor VIII activity test (which checks the amount and functioning of factor VIII, the factor that's missing or defective in the bleeding disorder hemophilia A) Because factor VIII circulates in the body attached to vWF, ...
How to draw blood from a vein?
If the blood is being drawn from a vein, the skin surface is cleaned with antiseptic, and an elastic band (tourniquet) is placed around the upper arm to apply pressure and cause the veins to swell with blood. A needle is inserted into a vein (usually in the arm inside of the elbow or on the back of the hand) and blood is withdrawn and collected in a vial. A chemical in the vial keeps the blood from clotting before the test begins.
How to help a child with a blood draw?
Tell your child to try to relax and stay still during the procedure, as tensing muscles and moving can make it harder and more painful to draw blood. It also may help for your child to look away when the needle is being inserted into the skin.
How long does it take for blood to go away?
Afterward, there may be some mild bruising, which should go away in a day or so.
Where is the needle inserted for blood test?
A needle is inserted into a vein (usually in the arm inside of the elbow or on the back of the hand) and blood is withdrawn and collected in a vial. A chemical in the vial keeps the blood from clotting before the test begins. After the procedure, the elastic band is removed.
What is von Willebrand disease?
This deficiency causes a condition referred to as von Willebrand disease (VWD). VWD is the most common inherited bleeding disorder. It is a group of conditions associated with prolonged bleeding due to deficient and/or defective VWF. VWD is separated into different types and sub-types, including:
What is the purpose of the Von Willebrand factor?
Von Willebrand factor (vWF or VWF) is a protein that is one of several components of the coagulation system that work together, and in sequence, to stop bleeding within the body. VWF testing measures the amount of the protein present in blood and determines how well the protein functions. Normally, when a blood vessel is damaged ...
What is the purpose of a VWF test?
Testing is used to help diagnose von Willebrand disease (VWD) and distinguish between the various types of VWD. VWF antigen – this test measures the amount of the VWF protein present in the blood. VWF activity (also called Ristocetin Cofactor) – this test determines whether the protein is functioning properly.
How does VWF affect clotting?
VWF further affects clotting by influencing the availability of coagulation factor VIII. VWF carries factor VIII in the blood, increases its half-life, and releases it as necessary. If the amount of functional VWF is insufficient, then platelet adherence and aggregation are affected, levels of factor VIII could be decreased, blood clot formation takes longer, and therefore bleeding is prolonged. This deficiency causes a condition referred to as von Willebrand disease (VWD).
What is VWF in blood?
Normally, when a blood vessel is damaged and bleeding begins, VWF forms an adhesive bridge between activated cell fragments called platelets and the injury site. This is followed by the clumping (aggregation) of platelets at the site and a series of actions referred to as activation of the coagulation cascade, resulting in the formation of a stable blood clot.
Where is VWF testing performed?
VWF testing must be performed in a laboratory and is often sent to a reference laboratory. It requires specialized equipment and interpretation.
Can you have VWD during menstruation?
It is possible, but many women who experience heavy bleeding during menstruation do not have VWD.
Why is it necessary to do a blood test for VWD more than once?
This is necessary because of the different types of the disorder. Because so many things affect the VWF level, it may be necessary to do the tests more than once. Some of the blood tests used to diagnose VWD are: Von Willebrand Factor Antigen – This test measures how much VWF is in the blood.
What is a Von Willebrand factor multimer?
Von Willebrand Factor Multimers – This test measures the different parts of the VWF protein. This can help determine which type of VWD someone has. It is typically done only after someone is confirmed to have VWD.
What is the PFA 100 test?
The PFA 100® result may be abnormal in VWD but may also be abnormal if there is a platelet dysfunction or anemia. Collagen Binding Assay – This test measures the binding of the VWF to collagen, one type of protein in the body. It is another test of the VWF function. PRINTER FRIENDLY VERSION.
How to raise VWF in children?
This can make VWD testing very difficult in children. Exercise – Exercise raises the VWF level in the blood. Infection – Having an active infection can increase the VWF level. A person’s blood type can also affect his or her VWF level.
Why is it so hard to test for VWD?
Testing can be difficult. Testing for VWD, especially Type 1, can be difficult because the level of VWF can be easy to raise temporarily. Some of the things known to affect VWF levels are: Pregnancy – VWF levels rise during pregnancy. Estrogen and some birth control pills – These can raise the VWF level in the blood.
Who can test for VWD?
Testing for VWD is best done by a specialist in bleeding disorders, such as the hematologist at a hemophilia treatment center (HTC). He or she will know how to perform and interpret the tests accurately. Your medical history is as important to the doctor as the laboratory tests. The doctor will want to know:
Can you have VWD for years?
Some people may have VWD for years and never know that they have it. This may be because they do not have symptoms or their symptoms are very mild. This can be especially true for men. They might only be diagnosed when having surgery or if a woman in their family is diagnosed. Other people may not be diagnosed because their doctors do not know ...
What are the different types of von Willebrand disease?
There are three main types of von Willebrand disease. Type 1 ( deficiency of vWF), the most common, is usually mild; type 2 (abnormal vWF) has several subtypes and is less common; and type 3 (absence of vWF), which is rare, is the most severe form 1.
What is the first step in evaluating a woman with suspected bleeding disorders?
The first step in the evaluation of women with suspected bleeding disorders involves obtaining a detailed medical history and performing a physical examination 7 10. Women with heavy menstrual bleeding since menarche, postpartum or surgical hemorrhage, plus additional bleeding symptoms, such as bruising, epistaxis, gingival bleeding, or family history of bleeding disorder are considered at risk. Box 1 includes a screening tool to help clinicians identify adult patients who may benefit from laboratory testing for disorders of hemostasis. Physical examination findings that may suggest a bleeding disorder include petechiae, ecchymoses, or other evidence of recent bleeding, although absence of these signs does not exclude the possibility of an underlying bleeding condition 7.
Can von Willebrand disease cause ovarian cysts?
The association of von Willebrand disease with other gynecologic problems––including ovarian cysts , endometriosis, and leiomyomas––is uncertain 1. Heavy menstrual bleeding or hemorrhagic ovarian cysts may be managed with combined hormonal contraceptives, which can address both the bleeding and the development of hemorrhagic cysts 12. For the acute presentation of a ruptured ovarian cyst, patients with von Willebrand disease may require surgical intervention for hemostasis.
Is postpartum hemorrhage higher in women with von Willebrand disease?
Notably, a large epidemiologic study reported that the risk of postpartum hemorrhage for women with von Willebrand disease was 50% higher than for women without a bleeding disorder 32.
Is von Willebrand disease inherited?
An autosomally inherited congenital bleeding disorder, von Willebrand disease involves a qualitative or quantitative deficiency of von Willebrand factor (vWF), a protein critical for proper platelet adhesion and protection against coagulant factor degradation. Dominant and recessive patterns of inheritance exist.
Is von Willebrand disease a common cause of heavy menstrual bleeding?
Conclusion. Von Willebrand disease is a common cause of heavy menstrual bleeding and other bleeding problems in women and adolescent girls. Obstetrician–gynecologists should include von Willebrand disease and other bleeding disorders in the differential diagnosis when evaluating patients with heavy menstrual bleeding, regardless of age.
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- Mild forms of von Willebrand disease can be difficult to diagnose because bleeding is common, and, for most people, doesn't indicate a disease. However, if your doctor suspects you have a bleeding disorder, he or she might refer you to a blood disorders specialist (hematologist). To evaluate you for von Willebrand disease, your doctor will likely a...