Bleeding Disorders affecting Women

Von Willebrand’s Disorder
Von Willebrand’s disorder (vWD) is the most common type of bleeding disorder. It affects about 1 % of the population. Unfortunately, bleeding symptoms are not always recognized as abnormal, so only a small number of affected individuals have been diagnosed.
In fact, it is not a single disorder, but a family of related disorders. All the different types are caused by a problem with the von Willebrand factor (VWF). This is a protein in blood which is necessary for proper blood coagulation, or clotting. It helps platelets stick to the wall of a damaged blood vessel. In addition, the VWF carries factor VIII, another protein important to blood clotting, through the bloodstream.
All types of VWD are inherited in an autosomal pattern (see how are Bleeding Disorders inherited).
There are 3 types of vWD:
Type 1 is the most common. About 75% of women with VWD have Type 1. With this type, the VWF works normally, but there is less of it than normal.
Type 2 is the next most common. The vWF protein is abnormal and does not work properly. Type 2 VWD includes several sub-types. Together, they make up 20-25% of VWD cases. In Type 2 VWD, the VWF molecule may be present in normal quantity, but it does not work properly. The VWF does not play its role in binding platelets to the wall of the blood vessel; sometimes it binds to platelets in the bloodstream instead of at the site of the broken vessel wall.
Type 3 vWD is the rarest and the most severe form. People with Type 3 vWD have almost no vWF. As vWF transports Factor VIII in the bloodstream, people with Type 3 vWD have very low levels of Factor VIII as well.
Bleeding disorders are rare and health professionals may not be familiar with them – or know that women and girls have bleeding symptoms too. Educating yourself about your bleeding disorder, getting a diagnosis and a treatment plan from a specialist Haemophilia Treatment Centre (HTC) and preparing for emergencies and procedures with your HTC – this will make a huge difference to your treatment experience.

Platelet Function Disorders
Platelet function disorders may affect as many as 1% of the population. The majority of these disorders are mild and many go undiagnosed. However, some types of platelet function disorders, such as Glanzmann’s Thrombasthenia, are more serious. Depending on the type of platelet function disorder, platelets may not stick to the walls of damaged blood vessels or form a proper surface so that other blood factors can form a clot at the site of an injury. The congenital platelet function disorders have variable inheritance patterns.
Immune Thrombocytopenia (ITP) is a blood disorder that causes the number of platelets in the blood to drop to such a low level that there is a risk of bleeding. The disorder is a spontaneously occurring condition that can affect both children and adults, and it is not hereditary. ITP can be short-lived or become ‘chronic’. Here, the term chronic does not necessarily mean that one never gets rid of the disease. This means that it can last for several or many years. Fortunately, most people with ITP can live a relatively normal life, taking into account the disease and following the necessary precautions.
Bleeding disorders are rare and health professionals may not be familiar with them – or know that women and girls have bleeding symptoms too. Educating yourself about your bleeding disorder, getting a diagnosis and a treatment plan from a specialist Haemophilia Treatment Centre (HTC) and preparing for emergencies and procedures with your HTC – this will make a huge difference to your treatment experience.

Haemophilia A and B and Carriers
Severe Haemophilia A (factor VIII deficiency, <1%) or Haemophilia B (factor IX deficiency, <1%) is extremely rare in women but it can happen. This is usually identified early through joint bleeding or other frequent bleeds.
Women can have factor levels less than 40% and should be consider as having mild (5-40%) or moderate haemophilia (1-5%). Often the term “carrier” is used to describe women, and this too often results in symptoms being dismissed.
The term “carrier” should be used and understood by health professionals to indicate the possibility to pass on a gene for haemophilia by a male or female.
Many carriers have a clotting level between 40-70% which would fit in the normal and do not usually suffer from severe bleeding, although they may still have difficulties from the most common symptom – heavy menstrual bleeding.
Factor VIII or FIX level less than 40% Factor VIII or FIX level more than 40%
Carrier identified through genetic testing or a family history Carrier and have a form of haemophilia with the associated bleeding, including heavy menstrual bleeding and post childbirth Carrier but have a form of haemophilia. There may still be still some issues with bleeding, including heavy menstrual bleeding and post childbirth

Bleeding disorders are rare and health professionals may not be familiar with them – or know that women and girls have bleeding symptoms too. Educating yourself about your bleeding disorder, getting a diagnosis and a treatment plan from a specialist Haemophilia Treatment Centre (HTC) and preparing for emergencies and procedures with your HTC – this will make a huge difference to your treatment experience.
Other clotting factor deficiencies
There are other bleeding disorders the are caused by deficiencies of other clotting proteins in the blood and these are often called Rare Bleeding Disorders. These are inherited in an autosomal recessive pattern (see How Bleeding Disorders are inherited).

Factor Other name Incidence
Factor I fibrinogen 1 in 1,000,000
Factor II prothrombin 1 in 1,000,000
Factor V Para Haemophilia 1 in 1,000,000
Combined F V
and F VIII 1 in 1,000,000
Factor VII 1 in 500,000
Factor X Stuart-Prower 1 in 500,0000
Factor XI Haemophilia C 1 in 100,000
Factor XIII 1 in 3,000,000