This book describes Von Willebrand Disease, Diagnosis and Treatment and Related Diseases
Von Willebrand disease is the most frequent hereditary bleeding disorder.
Causes
Von Willebrand disease is produced by a deficiency of the von Willebrand factor.
The Von Willebrand factor induces blood platelets to clump together and stick to the blood vessel wall, which is required for normal blood clotting
The vWF is a multi-meric glycoprotein encoded by gene map locus 12p13.31
It is produced in the endothelium and kept in Weibel-Palade bodies.
It has 2 major functions:
1.It helps in platelet plug formation by drawing circulating platelets to the site of damage.
2.It attaches to coagulation factor VIII stopping its clearance from the plasma.
It can be congenital or acquired:
1.Hereditary - 3 types and many sub-types.
2.Acquired - also called pseudo-von Willebrand's disease or platelet-type;
It is often found in lymphoproliferative or myeloproliferative disorders and can also be linked with:
a.Solid tumors,
b.Immunological and cardiovascular disorders
Symptoms
This differs depending on the deficiency:
1.Bleeding tendency from mucosa such as:
a.Epistaxis,
b.Menorrhagia
2.Spontaneous bleeding such as:
a.Internal or
b.Joint bleeding (most severe of cases).
3.Blood clots during childbirth
4.Death
Diagnosis
Von Willebrand disease may be difficult to diagnose.
Low von Willebrand factor levels and bleeding should not always indicate that the patient has von Willebrand disease.
Tests that may be done to diagnose this disease are:
1.Bleeding time
2.Blood typing
3.Factor VIII level
4.Platelet function analysis
5.Platelet count
6.Ristocetin cofactor test
7.Von Willebrand factor specific tests
Treatment

Treatment may be DDAVP (desamino-8-arginine vasopressin), a medicine to increase von Willebrand factor level and decrease the chances for bleeding.
DDAVP is not used for all types of von Willebrand disease.
Tests should be performed to determine what type of von Willebrand the patient has.
If the patient is going to have surgery, the doctor may give the patient DDAVP before surgery to see if the von Willebrand factor levels increase.
The drug Alphanate (antihemophilic factor) is also approved to reduce bleeding in people with the disease who must have surgery or any other invasive intervention.
Blood plasma or certain factor VIII preparations may also be given to reduce bleeding.
Patients should be educated on the bleeding risk.
Advice is given regarding drugs that must be stopped such as non-steroidal anti-inflammatory drugs and antiplatelet drugs.
Minor bleeding disorders, such as bruising or a brief nosebleed, may not need any specific treatment.
Treatments to obtain homeostasis in vWD are:
1.Tranexamic acid and
2.Desmopressin or
3.Concentrates having either high-purity vWF alone or
4.Intermediate-purity concentrates having factor VIII-vWF
Tranexamic acid is an agent that fights fibrinolysis.
It can be given:
1.Topically,
2.As a mouthwash,
3.Orally or
4.Intravenously,
5.As a treatment for minor bleeding or
6.Given before surgery, either on its own or
7.As an addition to desmopressin or concentrates.
In women with menorrhagia, combined oral contraceptives or progesterone-containing intrauterine contraceptives often provide significant medical benefit.
Platelet transfusions may be useful in some patients with disease resistant to other therapies.
For prevention in major surgery or...