Von Willebrand Disease Present

8/6/2019 Von Willebrand Disease Present

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VON WILLEBRANDVON WILLEBRAND

DISEASEDISEASE

HEMAMALINI A/P THANABALASINGAM

PRATIBA NAIR A/P RAMADHASAN


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INTRODUCTIONINTRODUCTION

Von Willebrand disease (VWD) is a bleeding disorder. Itaffects our blood's ability to clot. It is the most common

hereditary coagulation abnormality described in

humans.

In VWD, we either have low levels of a certain protein

in our blood, or the protein doesn't work the way it

should. The protein is called von Willebrand factor, and

it helps the blood clot.

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.


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Von Willebrand factor also carries clotting

factor VIII (8), another important protein thathelps your blood clot.

VWD is more common and usually milder

than hemophilia. VWD affects both males and

females, while hemophilia mainly affectsmales.

There are three major types of VWD :-

Type 1

-a low level of von Willebrand factor, and have

lower than normal levels of factor VIII. This is

the mildest and most common form of VWD.


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Type 2

- It is divided into subtypes: 2A, 2B, 2M, and

2N. Different gene mutations (changes)

cause each type, and each is treated

differently.

Type 3

- In type 3 VWD, usually have no von

Willebrand factor and low levels of factor VIII.Type 3 is the most serious form of VWD, but

it's very rare.


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What Causes vonWhat Causes von

WillebrandWillebrand Disease?Disease?Von Willebrand disease (VWD) is almost always

inherited.

Type 1 or type 2 VWD can be inherited if only one ofparents passes the gene on to children. Type 3 VWDcan be inherited only if both of parents pass the gene.

Some people have the genes for the disorder but don't

have symptoms. However, they still can pass the geneson to their children.

Some people develop a form of VWD later in life as aresult of other medical conditions. This form of VWD is

called acquired von Willebrand syndrome


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Signs and Symptoms of vonSigns and Symptoms of von

WillebrandWillebrand DiseaseDiseaseDepend on the type and severity of the disorder.Type 1 or type 2 VWD, may have the following mild-to-

moderate bleeding symptoms:-

Frequent, 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 intestinesor stomach


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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 thesymptoms listed before, as well as severebleeding episodes for no reason. These bleeding

episodes can be fatal if not treated right away.People who have type 3 VWD also may havebleeding into soft tissues or joints, causing severepain and swelling.


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DIAGNOSISDIAGNOSIS

Medical History

- Any bleeding from a small wound that lasted more than 15 minutes orstarted up again within the first 7 days following the injury.

-Any extended, heavy, or repeated bleeding that required medical

attention after surgery or dental extractions.

-Any bruising with little or no apparent trauma, especially if you could

feel a lump under the bruise.

-Any nosebleeds that occurred for no known reason and lasted more

than 10 minutes

-Any blood in your stools for no known reason.

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).


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Diagnostic Tests

A combination of blood tests recommended to diagnose the

disorder. These tests may include:

Von Willebrand factor antigen.- This test measures the amount of

von Willebrand factor in your blood.

Von Willebrand factor ristocetin (ris-to-SEE-tin) cofactor activity. -

This test shows how well your von Willebrand factor works.

Factor VIII clotting activity. -Some people who have VWD have low

levels of factor VIII activity, while others have normal levels.

Von Willebrand factor multimers. -This test is done if one or more ofthe first three tests are abnormal. It shows the structure of your

von Willebrand factor. The test helps your doctor diagnose whattype of VWD you have.

Platelet function test. -This test measures how well your platelets

are working.


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TREATMENT FOR VWDTREATMENT 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 usuallytaken by injection or nasal spray. It makes body release morevon Willebrand factor and factor VIII int bloodstream. DDAVPworks for most people who have type 1 VWD and for some whohave type 2 VWD.


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Von Willebrand factor replacement therapy is an infusion

of a concentrate of von Willebrand factor and factor VIII

into a vein in your arm. 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. They're mostly used to stop bleeding afterminor surgery, tooth extraction, or an injury. These

medicines may be used alone or together with DDAVP

and replacement therapy.


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Pregnancies and VWD

Pregnancies and VWD

Pregnancy can be a challenge for women who have VWD. Althoughblood levels of von Willebrand factor and factor VIII tend toincrease during pregnancy, women who have VWD can havebleeding complications during delivery. They also are likely to have

heavy bleeding for an extended time after delivery.

However, steps can be taken to lower the risk of complicationsduring pregnancy. Consult a hematologist and an obstetrician whospecialize in high-risk pregnancies before you become pregnant.

Before having any invasive procedure, such as amniocentesis (AM-

ne-o-sen-TE-sis), discuss with the doctor whether anything needsto be done to prevent serious blood loss.

During the third trimester,blood tests should be done to measurevon Willebrand factor and factor VIII to help plan for delivery.

With these precautions, most women who have VWD can havesuccessful pregnancies.


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Children and VWDChildren and VWD If a child has VWD that's severe enough to

pose a significant risk of bleeding, anyone

who is responsible for him or her should betold about the condition. This will help them

handle the situation if a child has an injury.


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FinallyFinally

VWD can't be cured, but it can be treated.

With the right treatment, people who have

VWD can lead normal, active lives.


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THANK YOUTHANK YOU

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Von Willebrand Disease Present