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Women and the Bleeding Disorders

Introduction
Many healthcare providers are not aware of the prevalence of bleeding disorders among females and may believe that prolonged bleeding occurs only in men. However, women can be affected by several different bleeding disorders such as Von Willebrand disease (VWD); platelet defects; Hemophilia A, B or  C; and other more rare clotting deficiencies, as well as disorders involving the fibrinolytic system.

Causes

 * 1) Von Willebrand disease
 * 2) Platelet function disorders
 * 3) Hemophilia A and B
 * 4) Other factor deficiencies

Von Willebrand disease
VWD is the most common inherited bleeding disorder, and it affects men and women equally. VWD follows an autosomal dominant inheritance pattern. Bleeding in VWD is predominantly mucocutaneous, and symptoms of nose bleeding, mouth bleeding and easy bruising are common. These can be dismissed by medical providers because they are difficult to quantify. Often evaluation for bleeding disorders is delayed until adolescence when menorrhagia occurs in the young woman. Women with VWD often have both prolonged and profuse menstrual bleeding. Menses may continue for 10-14 days, instead of the average 5-day cycle that most women experience. Changing pads or tampons as often as every hour is a common experience for the woman with VWD. Using double protection and flooding of menstrual blood through the protection and onto clothing is often reported.

Platelet function disorders
Platelet function disorders may be as common as von Willebrand disease. However, because many of these disorders are mild, many women go undiagnosed.

Some types of platelet function disorder, such as Glanzmann's thrombasthenia, can be life-threatening.

Depending on the type of platelet function disorder:
 * Platelets do not stick to the walls of damaged blood vessels
 * Platelets do not clump together at the site of the injury to the blood vessel
 * Platelets do not form a proper surface so that other blood factors can make a fibrin clot.

Hemophilia A and B
Hemophilia A (factor VIII deficiency) affects 1 in 5000 males. Hemophilia B (factor IX deficiency) affects 1 in 25,000 males. Severe hemophilia in women is extremely rare. However, women are carriers of hemophilia and many women who are carriers have bleeding problems. The genes responsible for producing factors VIII and IX, the defective genes in hemophilia, are situated on the X chromosome. This makes hemophilia a sex-linked genetic disorder. Carriers have one normal X chromosome and one abnormal X chromosome. The normal X chromosome produces a certain amount of factor VIII or IX clotting factor. This protects carriers from the most severe form of hemophilia in which the level of clotting factor is less than 1%. However, the variation in clotting factor levels among carriers is very wide. In some carriers the level is much below normal. In other carriers, the level is closer to normal. This is because the two X chromosomes, one of which carries the hemophilia gene, are not equally functional. If the hemophilia X chromosome happens to be functional in most cells, then the carrier will have a very low level of clotting factor activity.

Many carriers have a clotting level between 30% and 70% of normal and do not usually suffer from severe bleeding, although they may suffer from the most common symptom - heavy menstrual bleeding.

However, some carriers have less than 30% of the normal level of factor VIII or IX. These women are considered to have mild hemophilia. It is not known how many carriers of hemophilia A and B have bleeding problems. Estimates vary up to 60%.

Other factor deficiencies
Factor XI deficiency is a very rare bleeding disorder. Also called Hemophilia C, it affects approximately 1 in 100,000 people in the United States.Those with Ashkenazi Jewish heritage are found to be more frequently affected, leading to higher incidence of the condition in regions with large populations of individuals with eastern European or Israeli heritage. Factor XI deficiency is increasingly being found in persons from all ethnic groups, as well as in those with no family history of bleeding. (7) Factor XI deficiency follows an autosomal dominant inheritance pattern, meaning that males and females are equally affected. Other rare factor deficiencies may be found in women. Factor V, Factor VII, Factor X and Factor XIII deficiencies may all produce excessive bleeding.

Symptoms

 * Heavy or prolonged menstrual bleeding (menorrhagia)
 * Abnormal bleeding after childbirth
 * Easy bruising
 * Frequent and prolonged nosebleeds
 * Prolonged bleeding after dental procedures
 * Abnormal bleeding after surgery or trauma

Diagnosis
For von Willebrand disease, the following tests permit an accurate diagnosis:

For platelet function disorders, the following tests permit an accurate diagnosis:

For hemophilia A and B, the following tests permit an accurate diagnosis:

Because the other clotting factor deficiencies are so rare, tests are first done to rule out von Willebrand disease, platelet function disorders, and hemophilia A and B as the cause of bleeding. If one of the rare factor deficiencies is suspected, a laboratory assay is done to measure the level of the specific clotting factor.