VON WILLEBRAND DISEASE

HEMAMALINI A/P THANABALASINGAM PRATIBA NAIR A/P RAMADHASAN

It affects our blood's ability to clot. and it helps the blood clot. € In VWD. or the protein doesn't work the way it should.INTRODUCTION € Von Willebrand disease (VWD) is a bleeding disorder. . The protein is called von Willebrand factor. It is the most common hereditary coagulation abnormality described in humans. € Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot when blood vessels injured and start to bleed. we either have low levels of a certain protein in our blood.

There are three major types of VWD :Type 1 -a low level of von Willebrand factor. . VWD affects both males and females. This is the mildest and most common form of VWD. while hemophilia mainly affects males.Von Willebrand factor also carries clotting factor VIII (8). another important protein that helps your blood clot. and have lower than normal levels of factor VIII. VWD is more common and usually milder than hemophilia.

.In type 3 VWD. usually have no von Willebrand factor and low levels of factor VIII.Type 2 . 2M. but it's very rare.It is divided into subtypes: 2A. 2B. and 2N. Different gene mutations (changes) cause each type. Type 3 . and each is treated differently. Type 3 is the most serious form of VWD.

€ Type 1 or type 2 VWD can be inherited if only one of parents passes the gene on to children. € Some people have the genes for the disorder but don't have symptoms. they still can pass the genes on to their children. However. € Some people develop a form of VWD later in life as a result of other medical conditions.What Causes von Willebrand Disease? € Von Willebrand disease (VWD) is almost always inherited. Type 3 VWD can be inherited only if both of parents pass the gene. This form of VWD is called acquired von Willebrand syndrome .

may have the following mild-tomoderate bleeding symptoms:-      Frequent.Signs and Symptoms of von Willebrand Disease € Depend on the type and severity of the disorder. € Type 1 or type 2 VWD. large bruises from minor bumps or injuries Frequent or hard-to-stop nosebleeds Extended bleeding from the gums after a dental procedure Heavy or extended menstrual bleeding in women Blood in your stools from bleeding in your intestines or stomach .

causing severe pain and swelling. People who have type 3 VWD also may have bleeding into soft tissues or joints. as well as severe bleeding episodes for no reason. . Blood in your urine from bleeding in your kidneys or bladder  Heavy bleeding after a cut or other accident  Heavy bleeding after surgery €People who have type 3 VWD may have all of the symptoms listed before. These bleeding episodes can be fatal if not treated right away.

or repeated bleeding that required medical attention after surgery or dental extractions. heavy. -Any nosebleeds that occurred for no known reason and lasted more than 10 minutes -Any blood in your stools for no known reason. . -Any bruising with little or no apparent trauma. -Any extended.Any bleeding from a small wound that lasted more than 15 minutes or started up again within the first 7 days following the injury. Physical Exam .To look for unusual bruising or other signs of recent bleeding and also look for evidence of liver disease or anemia (a low red blood cell count).DIAGNOSIS Medical History . especially if you could feel a lump under the bruise.

. Von Willebrand factor ristocetin (ris-to-SEE-tin) cofactor activity.. Platelet function test. while others have normal levels. This test shows how well your von Willebrand factor works. -This test measures how well your platelets are working.Diagnostic Tests A combination of blood tests recommended to diagnose the disorder. The test helps your doctor diagnose what type of VWD you have. Von Willebrand factor multimers. -This test is done if one or more of the first three tests are abnormal. Factor VIII clotting activity.This test measures the amount of von Willebrand factor in your blood. -Some people who have VWD have low levels of factor VIII activity. It shows the structure of your von Willebrand factor. These tests may include: Von Willebrand factor antigen.

TREATMENT FOR VWD Treatment for von Willebrand disease (VWD) is based on the type of VWD and how severe it is. . Medicines are used to: Increase the release of von Willebrand factor and factor VIII into the bloodstream Replace von Willebrand factor Prevent the breakdown of blood clots Control heavy menstrual bleeding in women Specific Treatments Desmopressin (DDAVP) is a synthetic hormone that usually taken by injection or nasal spray. DDAVP works for most people who have type 1 VWD and for some who have type 2 VWD. It makes body release more von Willebrand factor and factor VIII int bloodstream.

or an injury. . They're mostly used to stop bleeding after minor surgery. tooth extraction. This treatment may be used if : Can't take DDAVP or need extended treatment Have type 1 VWD that doesn't respond to DDAVP Have type 2 or type 3 VWD Antifibrinolytic medicines help prevent the breakdown of blood clots. These medicines may be used alone or together with DDAVP and replacement therapy.Von Willebrand factor replacement therapy is an infusion of a concentrate of von Willebrand factor and factor VIII into a vein in your arm.

Fibrin glue is medicine that's placed directly on a wound to stop bleeding Women who have VWD also may be treated with oral contraceptives. thus reducing menstrual blood loss. intrauterine devices. . or a procedure that destroys the lining of the uterus.

blood tests should be done to measure von Willebrand factor and factor VIII to help plan for delivery. Before having any invasive procedure. discuss with the doctor whether anything needs to be done to prevent serious blood loss. Although blood levels of von Willebrand factor and factor VIII tend to increase during pregnancy. most women who have VWD can have successful pregnancies. However. . steps can be taken to lower the risk of complications during pregnancy. They also are likely to have heavy bleeding for an extended time after delivery. Consult a hematologist and an obstetrician who specialize in high-risk pregnancies before you become pregnant.Pregnancies and VWD Pregnancy can be a challenge for women who have VWD. women who have VWD can have bleeding complications during delivery. With these precautions. such as amniocentesis (AMne-o-sen-TE-sis). During the third trimester.

anyone who is responsible for him or her should be told about the condition. This will help them handle the situation if a child has an injury. .Children and VWD If a child has VWD that's severe enough to pose a significant risk of bleeding.

people who have VWD can lead normal. With the right treatment. but it can be treated. active lives. .Finally VWD can't be cured.

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