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Von Willebrand disease

Von Willebrand's disease (vWD) is the most common hereditary coagulation abnormality described in humans. It arises from a qualitative or quantitative deficiency of von Willebrand factor (vWF), a multimeric protein that is required for platelet adhesion. It is known to affect humans and, in veterinary medicine, dogs. more...

VACTERL association
Van der Woude syndrome
Van Goethem syndrome
Varicella Zoster
Variegate porphyria
Vasovagal syncope
VATER association
Velocardiofacial syndrome
Ventricular septal defect
Viral hemorrhagic fever
Vitamin B12 Deficiency
VLCAD deficiency
Von Gierke disease
Von Hippel-Lindau disease
Von Recklinghausen disease
Von Willebrand disease


The various types of vWD present with varying degrees of bleeding tendency. Severe internal or joint bleeding is rare (only in type 3 vWD); bruising, nosebleeds, heavy menstrual periods (in women) and blood loss during childbirth (rare) may occur.


When suspected, blood plasma of a patient needs to be investigated for quantitative and qualitative deficiencies of vWF. This is achieved by measuring the amount of vWF in a vWF antigen assay and the functionallity of vWF with a glycoprotein (GP)Ib binding assay, a collagen binding assay or, a ristocetin cofactor (RiCof) activity assay. Factor VIII levels are also performed as factor VIII is bound to vWF which protects the factor VIII from rapid break down within the blood. Deficiency of vWF can therefore lead to a reduction in Factor VIII levels. Normal levels do not exclude all forms of vWD: particularly type 2 which may only be revealed by investigating platelet interaction with subendothelium under flow (PAF), a highly specialistic coagulation study not routinely performed in most medical laboratories. A platelet aggregation assay will show an abnormal response to ristocetin with normal responses to the other agonists used. A platelet function assay (PFA) will give an abnormal collagen/adrenaline closure time but a normal collagen/ADP time. Type 2N can only be diagnosed by performing a "factor VIII binding" assay. Detection of vWD is complicated by vWF being an acute phase reactant with levels rising in infection, pregnancy and stress.

Other tests performed in any patient with bleeding problems are a full blood count (especially platelet counts), APTT (activated partial thromboplastin time), prothrombin time, thrombin time and fibrinogen level. Testing for factor IX may also be performed if hemophilia B is suspected. Other coagulation factor assays may be performed depending on the results of a coagulation screen.

Classification and types


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Diagnosis key to treating von Willebrand disease - Bleeding - medical research
From USA Today (Society for the Advancement of Education), 2/1/03

Although yon Willebrand disease (VWD) is the most-commonly inherited bleeding disorder, affecting approximately 3,000,000 Americans, few people--including physicians--are familiar with the condition. This lack of awareness may contribute to the low rate of diagnosis (It is estimated that over 99% of those with VWD have not been diagnosed.) Proper diagnosis provides a name and more importantly, an explanation for what makes someone with VWD "different." Contrary to what victims may have been told, their symptoms are not imaginary and knowing the cause allows them to come to terms with and take control of the, condition.

Unlike hemophilia, which as very rare in women, VWD affects men and women en equal numbers. However, its impact on females can be greater, especially when undiagnosed, due to their physiology. For women with VWD. menstrual periods are unusually long and heavy, often lasting longer than seven days and requiring the changing of tampons or pads more frequently than every two hours. If misdiagnosed, this can lead to unnecessary medical procedures, including hysterectomies, D&Cs (dilation and curretage), and endometrial ablation, as well as prolonged bleeding following childbirth.

Besides the medical implications, VWD can have a profound impact on women's daily lives--from self-esteem to intimacy--that makes appropriate diagnosis and treatment even more urgent Females who suffer from it regularly experience excessive or prolonged menstruation (menorrhagia) As a result, common experiences for many women--a fear of bleeding through clothes, playing sports, or a trip to the beach--are heightened. Menorrhagia also contributes to missed days of school or work, interrupting the education of girls and careers of women with VWD.

Unnecessary hysterectomies are frequent in those with misdiagnosed VWD because menorrhagia is misunderstood by many health care professionals. This procedure frequently happens to women as early as in their 20s and 30s. A fair proportion of women with undiagnosed VWD have undergone a hysterectomy for control of menorrhagia. One study found that seven percent of those who had a hysterectomy to control heavy menstrual bleeding were later diagnosed with VWD and therefore might have been able to be treated without any surgery.

Not only is there physical and psychological trauma with an unnecessary hysterectomy, but the procedure itself can be dangerous and potentially fatal to women with an undiagnosed bleeding disorder. Those who survive still must come to terms with the implications a hysterectomy has on their identity and possible plans for motherhood.

VWD can also have physical and psychological effects on females' intimate relationships and sexuality. Teenagers with VWD may lack the confidence to enjoy dating. As they mature and enter more-intimate relationships, women with VWD need to consider disclosing their symptoms (or, if they know it, diagnosis) with partners, which poses a risk of misunderstanding or even rejection These issues are compounded by physiology In many women, penetrative sexual intercourse can lead to vaginal tearing, which is normal. However, for those with VWD, this tearing can lead to excessive vaginal bleeding. As a result, sexual intercourse is often accompanied by, or results in, excessive bleeding. It is not surprising, then, that VWD--especially when undiagnosed--can lead to a decreased desire for sexual intercourse, diminished sexual pleasure, or relationship problems.

While a VWD diagnosis can bring its own challenges, because this disease is treatable, most women come to accept it as one part of who they are and live a full life while managing their condition. Equally important, a diagnosis may help women with VWD receive expert medical care--e.g., through Federally funded hemophilia centers, which serve the VWD community and provide state-of-the-art treatment from experienced specialists in bleeding disorders. In addition to medical care, these centers offer education and support, and many have social workers on site. Support and peer groups provide an opportunity to learn from others who are facing similar experiences.

COPYRIGHT 2003 Society for the Advancement of Education
COPYRIGHT 2003 Gale Group

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