Von willebrand's disease

1. VON WILLEBRAND'S DISEASE
By,
Ms. Ekta S Patel.

2. “× Inherited (autosomal dominant)
or acquired bleeding disorder
characterized by decreased
level of von Willebrand factor
and prolonged bleeding time.

3. Etiology
× von Willebrand factor synthesized
in vascular endothelium,
megakaryocytes and platelets;
enhances platelet adhesion as first
step in clot formation, also acts as
carrier of factor VIII in blood.

4. × Acquired form is rare, generally
appears late in life, typically in
association with lymphoma,
leukemia, multiple myeloma, or
autoimmune disorder.

5. risk factor
× The main risk factor for von
Willebrand disease is having a
family history of it.
× A parent can pass the abnormal
gene for the disease to his or her
child.

6. × Most cases are "autosomal
dominant inherited" disorders,
which means only need an
abnormal gene from one parent to
be affected.
× If patient have the gene for von
Willebrand disease, patient have a
50 percent chance of transmitting
this gene to offspring.

8. × The most severe form of the
condition (type 3) is "autosomal
recessive," which means both of
parents have to pass an abnormal
gene.

10. Von Willebrand disease has several types:
× Type 1.
× In this most common form of von
Willebrand disease, levels of von
Willebrand factor are low. In some
people, levels of factor VIII also are
low. Signs and symptoms are
usually mild.

11. × Type 2.
× In this type, which has several
subtypes, the von Willebrand factor
you do have doesn't function
properly. Signs and symptoms tend
to be more significant.

12. × Type 3.
× In this rare type, von Willebrand
factor is absent and levels of factor
VIII are low. Signs and symptoms
may be severe, such as bleeding
into the joints and muscles.

13. × Acquired von Willebrand
disease: This type isn't inherited
from parents. It develops later in
life.

14. Symptoms
× Many people with von Willebrand
disease don't know it because the
signs are mild or absent.

15. × The most common sign of the
condition is abnormal bleeding. The
severity of the bleeding varies from
one person to another.

16. × Excessive bleeding from an injury
or after surgery or dental work
× Nosebleeds that don't stop within
10 minutes
× Heavy or long menstrual bleeding
× Blood in urine or stool
× Easy bruising or lumpy bruises

17. Signs and symptoms of von Willebrand disease in
women
× Signs and symptoms of a heavy
period that may indicate von
Willebrand disease include:
× The presence in menstrual flow of
blood clots greater than 1 inch (2.5
centimeters) in diameter

18. × The need to change menstrual pad
or tampon more often than hourly
× The need to use double sanitary
protection to control menstrual flow
× Symptoms of anemia, including
tiredness, fatigue or shortness of
breath

19. D/E
× Von Willebrand factor antigen:
× This test determines the level of
von Willebrand factor in blood by
measuring a particular protein.

20. × Ristocetin cofactor activity:
× This test measures how well the
von Willebrand factor works in
clotting process.
× Ristocetin, which is an antibiotic, is
used in this laboratory testing.

21. × Factor VIII clotting activity.
× This test shows whether patient
have abnormally low levels and
activity of factor VIII.

22. × Von Willebrand factor multimers:
× This test evaluates the specific
structure of von Willebrand factor in
blood, its protein complexes
(multimers) and how its molecules
break down.
× This information helps identify the
type of von Willebrand disease you
have.

23. Management
× Replacement of von Willebrand's
factor and factor VIII using clotting
factor concentrates (Alphanate,
Humate-P).

24. × Antifibrinolytic medications (Amicar,
tranexamic acid) to stabilize clot
formation before dental procedures
and before minor surgery.

25. × Desmopressin acetate (DDAVP), a
synthetic analogue of vasopressin,
may be used to manage mild to
moderate bleeding.

26. × Estrogen and progesterone
stimulate production of von
Willebrand's factor and Factor VIII
and may be particularly helpful in
control of menorrhagia.

27. Complications
× Anemia.
× Swelling and pain.
× Death from bleeding.