Von the deficiency of the Von- Willebrand factor

Von Willebrand disease

Von-Willebrand is a bleeding disorder which affects the clotting mechanism in the body. During injury excess of bleeding occurs which may lead to internal organ damage rarely it may also lead to death. The clotting mechanism is altered due to the deficiency of the Von- Willebrand factor which is a key factor in the clotting process and they also carry the clotting factor VIII which is also an important clotting factor. The Von-Willebrand factor sticks to the platelets and it forms a clot when the blood vessels are injured and the bleeding is arrested or stopped. 1

Types of von Willebrand Disease 1

The three major types of VWD are Type 1, Type 2, and Type 3.

Type

Levels of Von-willebrand factor

Levels of factor VIII

Condition

I

Low

Low

Common
(60%-80%)

II

Low-altered function

Low

Moderate
(15%-30%)

III

No factor present

Low

Rare
(5%-10%)

Acquired Von willebrand disease

 
Low

 
Low

Acquired in later stage of life Rare

 

CLINICAL SYMPTOMS 2

·         Excessive bleeding during injury or surgery or dental work

·         Excessive Nosebleeds

·         Heavy menstrual bleeding

·         Blood in urine or stools

·         Bruising

DIAGNOSIS

·         Von Willebrand factor antigen: The level of Von Willebrand factor is measured.

·         Ristocetin cofactor activity: Ristocetin is an antibiotic used in measuring Von Willebrand factor works in the clotting process

·         Factor VIII clotting activity:  low levels and activity of factor VIII are measured

·         Von Willebrand factor multimers: this helps in evaluating the specific structure of von Willebrand factor in blood, its protein complexes (multimers) and its molecules break down. This helps to identify the type of Von Willebrand disease.

·         Platelet function test: measures the functions of the platelets. 1

TREATMENT 2

CLASS

DRUGS

BRAND NAME

Anti-diuretic hormones

Desmopressin

DDAVP,Minirin,Stimate,Noctiva

Antifibrinolytic or Clot-stabilizing medications

Aminocaproic acid 
Tranexamic acid

Amicar
Cyklokapron, Lysteda

Replacement therapies

Combination of von Willebrand factor and factor VIII

Humate-P

Contraceptives

Levonorgestrel
Ethnyl estrodiol
 

Beyaz, Safyral
Altavera , Amethyst, Aviane, Canrese