BLOOD CLOTTING DISORDER

Von willebrand disease

Presented by E.KEERTHANA B.Sc., M.Sc.,Under the Guidance of Dr. Mrs. S.SUGANTHI M.Sc., M.Phil., Ph.D.,

BLOOD CLOTTING MECHANISM

VON WILLEBRAND DISEASE Also call VWF,antihemophilia A bleeding disorder caused by low level of clotting

protein in the blood The condition is most often inherited but in rare

cases may develop later in life Is a hereditary familial disease due to a deficiency

of antihemophilic globulim and capillary defect, marked by severe epistaxis and bleeding from the gums and denitalia called antihemophilia

The cause of von willebrand disease deficiency in or impairment of a protein called von willebrand factor an important compounds in blood clotting process.

VON WILLEBRAND DISEASEThere are three types, TYPE 1 TYPE 2 There are 4 subtypes

in type 2 ,there are 2A,2B,2M and 2N.

TYPE 3

VON WILLEBRAND DISEASETYPE1 Most common type Small amount of

VWF is present in the body to help clot blood

In individual with VWF level <0.3IU/ml

Automosomal dominant

TYPE2 VWF have normal

level of VWF but there are defect in the factor function and structure.

It s divided into subtypes depending on severity

2A,2B,2M,and 2N

VON WILLEBRAND DISEASE

VON WILLEBRAND DISEASETYPE3 Most dangerous

type Because no VWF at

all presend in the boby.

So platelet cannot clot.

Type 3 is difficuit to stop.

VON WILLEBRAND DISEASECAUSES The usual cause of

vonwillebrand disease is an inherited defect in the gene that control von willebrand factor, a protein that plays a key role in blood clotting process.

When VWF is scare/or not funtioning properly because of stuctural abnormalities

Small blood cells called platelets cannot stick together properly nor can they attach themselves normally to the blood vessels walls when an injujury has occurred.

The results uncontrolled bleeding may persist

Von willebrand factor carries an additional substance called factor V111 that help stimulate clotting.

VWD have low level of factoe V111

Rarely ,vwd can develop later in life ,the abnormal gene from the parent know as acquired von willebrand diseases.

The exact cause of VWC is not clear.

It may be an autoimmune disease or it may be linked to slow thyroid gland(hypothyroidism) or certain medication such as depakene or antibiotic ciprofloxcin(cipro)

VON WILLEBRAND DISEASECOMPLICATIONANEMIA Excess menstural bleeding Iron deficiency anemiaSWELLING AND PAIN Abnormal bleeding occurs

in the joints or soft tissues leads to swelling and severe pain can result .

DEATH FROM BLEEDINGWhen abnormal bleeding cant

be controlled it can become life threatiening .

VON WILLEBRAND DISEASERISK FACTOR Autosomal dominant

inheritance pattern Autosomal recessive

inheritance pattern A family history of VWD is

the leading risk factor. A parent can pass the abnormal gene for the disease to his or her child

Most cause are autosomal dominant inheritance disorder,ie.abnormal gene from one parent to be affected.

The most severe form of the condition (type3) is autosomal recessive, which means both of parent have too pairs of abnormal gene .

VON WILLEBRAND DISEASESYMPTOMS Bleeding Blood in urine Easy bruising Excessive bleeding

during childbirth Heavy or prolonged

peroids

Mouth bleeding Nose bleeding Tendency to bleed

easily Dental procedure Irregugular uterine

bleeding

VON WILLEBRAND DISEASEDIAGOSTIC TEST Bleeding time blood

typing Factor V111 level Platelet function

analysis Platelet count Ristocetin cofactor

test VWF specific test

DRUGSTO BE AVOID Aspirin and Non-steroidal

anti inflammatory drugs(NASIDs)

Both the drugs can increase bleeding.

TO BE TAKEN DDAVP(desamino –

gamma-arginine vasopressin)

Alphanate(antihemophillic factor)

VON WILLEBRAND DISEASE

VON WILLEBRAND DISEASEREFERENCE1. Physiology by J.L.jain2. Essential of physiology by sembulingam3. http://www.mayoclinic.org/diseases-condi

tions/von-willebrand-disease/basics/definition/con-20030195

4. http://www.healthline.com/health/von-willebrand-disease#Overview1

5. https://medlineplus.gov/ency/article/000544.htm