Von Willebrant's disease is one of the most common bleeding problems in the world. According to statistics, Centers for Diseases Control and Prevention (CDC) represents 1% of the total population with the same distribution among men and women.
Clinically, von Willebrant's disease is a heterogeneous disorder of the hemostasis system caused by quantitative or qualitative insufficiency of the Willebrant factor.
Leebeek FW, Eikenboom JC. N Engl J Med 2016;375:2067-2080.
There are three main types of classification:
And type is a partial quantitative failure of vWF
Type II is a vWF qualitative failure
Type III is the complete absence of vWF
Type II is further subdivided into 4 subtypes (2A, 2B, 2N, 2M) - characterized by functional or structural disorders of the monomers of the Villebrant factor.
According to a study conducted in France among 670 families with von Willebrant's disease, the following distribution was determined by type:
And type - 25%
Type II - 66%
Type III - 8%
Indeterminate type - 1%
Signs and symptoms worth examining:
Spontaneous bleeding, which begins without trauma or other provocative factors and lasts longer than 5 - 10 minutes, often occurs (more than 5 times / year); support procedures are required to stop (jute, padding, pinching)
The appearance of bruises, ecchymoses, petechiae with the slightest mechanical irritation, which often appear (3-4 times a month), are spilled and do not rise above the skin surface
Abundant menstrual periods in women (the need to change the pad more than once every 2 hours) and accompanied by manifestations of anemia.
Bleeding, longer than normal (more than 5 minutes) after minor surgery, childbirth, tooth extraction, trauma, and atypical - the appearance of blood in the stool, urine, bleeding into the internal organs in the event of any other cause.
Main treatment options: desmopressin acetate (intravenous, subcutaneous, nasal spray), recombinant vWF, vWF / FVIII concentrate, antifibrinolytic agents (tranexamic acid, aminocaproic acid, etamzilate), combined contraceptives
Particular attention should be paid to women with von Willebrant's disease at childbearing age, during pregnancy and childbirth:
Women who have any bleeding problems (including heavy menstruation, spontaneous uterine bleeding) should tell their doctor before planning pregnancy and delivery.
In any case, a team of specialists (obstetrician-gynecologist, hematologist, surgeon, anesthetist) should work with such a woman.
Weigh the need for invasive procedures during pregnancy (eg, amniocentesis) from a “risk-benefit” point of view to avoid possible bleeding
During the third trimester, constantly monitor the level of vWF and factor VIII for birth planning
Inform the anesthesiologist about the risks of bleeding and discuss the need and possible methods of pain management in childbirth
Discuss the risks of inheriting Von Wlebrant's disease in the future toddler and how to diagnose them
During the third trimester of pregnancy, the number of coagulation factors in the woman's body is usually increased in order to minimize blood loss during childbirth. However, even after the birth of a baby, the level of these factors decreases sharply, which can cause bleeding in the early postpartum period, so such women should be closely monitored by a team of specialists and medical staff.
Information from https://www.cdc.gov/