Von Willebrand Disease


Von Willebrand disease ( VWD) is a genetically developed bleeding disorder caused by abnormality of von Willebrand factor .Von willebrand disease is an common hereditary bleeding disorder.

Von Willebrand disease , first described by Erik Adolf Von Willebrand in 1926 , being an congenital bleeding disorder it is characterized by frequent bleeding (epistaxis ) and menorrhagia .It involves both quantitative and qualitative deficiency of Von Willebrand factor.

The main function of VWF is to bind platelets ( small cell fragments ) in blood .This abnormal gene is not on X-chromosome ( X-chromosome is sex related chromosome ) but is present on autosome ( it is an regular chromosome ) .

In some families VWD appears as dominant condition due to abnormal gene from one parent , but in other case VWD may be inherited as a recessive with abnormal gene from both parents.Age / Sex groups affected by Von Willebrand Disease

There are no specific age groups identified, one can be diagnosed at any age.

Races affected by Von Willebrand Disease

Individuals of any race can be affected.

Classification of Von Willebrand Disease

Type Ι:
This is the most common and mildest form of Von willebrand disease. Level of Von willebrand factor is lower than the normal.

Type ΙΙ:
This type is due to abnormal Von willebrand factor. It is further classified into two types:

 Type ΙΙa:
In this type the level of Von willebrand factor is reduced, as is the ability of platelets to clump together.

 Type ΙΙb:
In this type Von willebrand factor itself id defective, the ability of platelets to clump together is actually increases.

Type ΙΙΙ:
This is severe type in which a total absence of Von willebrand factor may be countered.

Causes of Von Willebrand Disease

Von Willebrand disease is mainly caused by hereditary defect in gene controlling Von Willebrand factor .In this people have low levels of factor VIII , as Von Willebrand also carries an additional substance called factor VIII which stimulates clotting .

Risk factors
• Family history
• Parents passing the abnormal gene to his/her child

Von Willebrand Disease Signs and Symptoms

Abnormal bleeding in form of –
• Recurring and spontaneous nose bleed
• Gums bleeding
• Increased menstrual flow
• Blood in stool/urine
• Women –having heavy menstrual flow lasting for more than one week .Women has to use double sanitary protection.
• Anemia , fatigue , tiredness , shortness of breath

Prolonged bleeding from small injuries
Heavy  prolonged bleeding following surgery
Prolonged bellowing dental procedure
Blood in stool, bleeding ulcer
Heavy bleeding during of following childbirth

Investigations of Von Willebrand Disease

• Complete medical history.
• Complete genetic history, physical examination
• Bleeding time
• Factor VΙΙΙ level.
• Platelet count.
• Von Willebrand Factor antigen tests.
Complete blood count
Prothrombin time
Partial Thromboplastin time
Thrombin time

Prognosis of Von Willebrand Disease

Bleeding may decrease during pregnancy. Women who have this condition usually do not have excessive bleeding during childbirth.
The disease is passes down through families.
Genetic counseling may help prospective parents understand the risk to their children.

Complications of Von Willebrand Disease

In case patient has to go under knife he/she should inform prior to the healthcare provider about the hereditary or medical history of Von willebrand disease.

Diet / management of Von Willebrand Disease

• Avoid taking analgesics/ antidepressants without doctor’s recommendation as some of them causes thinning of blood.
• Staying physically active.
• Weight management.

Treatment of Von Willebrand Disease

• Replacement therapies – In this infusion of concentrated blood -clotting factors contaning Von Willebrand factor and factor VIII are given .
• Contraceptives – In women for controlling heavy bleeding during menses .
• Clot stabilizing medicines for slowing the breakdown of clotting factors

Differential diagnosis of Von Willebrand Disease

•Factor X

•Factor XI deficiency

•Hemophilia A

Homeopathic treatment for Von Willebrand Disease

Crotalus horridus –
• Low septic state, blood decomposition
• Epistaxis , blood black and stringy
• Intestinal haemorrhage
• Blood oozing from rectum

Hamamelis –
• Haemorrhages , profuse epistaxis
• Haematemesis of black blood
• Menses dark ,profuse , uterine haemorrhage.

Lachesis –
• Sensitive nostrils with epistaxis
• Haemorrhage from bowels
• Left ovary swollen
• Gums swollen , bleeding and spongy

Thlaspi bursa pastoris –
• Haemorrhage from uterine fibroid
• Bleeding in nasal surgeries
• Too frequent and copious menses

Phosphorus –
• Bleeding , epistaxis , polypi always bleeding
• Swollen and easily bleeding gums discharge of blood from rectum
• Menses lasts long
• Haemorrhage from uterus

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