Hematology

Hematology facts

In people with bleeding disorders, the blood clotting process does not work properly. As a result, people may bleed for longer than normal and may have bleeding into joints, muscles or other parts of the body.

In hemophilia A and von Willebrand disease (VWD), specific proteins required for the blood clotting process are missing or do not work as they should.

Signs of hemophilia include large bruises, bleeding into muscles and joints, sudden bleeding inside the body for no clear reason, or bleeding for a long time after a cut or wound.

What is hemophilia A?

Hemophilia A is a congenital bleeding disorder resulting from a deficiency of coagulation FVIII. Hemophilia A is a rare bleeding disorder, affecting approximately one in 5,000 male births and approximately 20,000 have hemophilia A in the United States. It is usually inherited from the parents (called congenital hemophilia A), although it can also develop without being inherited (called sporadic or acquired hemophilia A). Hemophilia A is diagnosed by taking a blood sample and measuring the level of FVIII activity.

Hemophilia A varies in severity and is classified as mild, moderate or severe, depending on the amount of FVIII the person has. People with severe hemophilia A have less than 1% of the normal amount of FVIII.

What is von Willebrand Disease?

People with von Willebrand disease (VWD) have a problem with a protein called von Willebrand factor (VWF). When a blood vessel is injured and bleeding occurs, VWF helps cells in the blood (called platelets) to form a clot to stop the bleeding. People with VWD have reduced levels of VWF, or their VWF does not work normally, so it takes longer for blood to clot and for bleeding to stop.

VWD is the most common bleeding disorder and is generally less severe than other bleeding disorders. It is estimated that up to 1% of the world’s population suffers from VWD but, because many people have mild symptoms, only a small number of them know they have VWD. For most people with VWD, the disorder causes little or no disruption to their lives except when they bleed heavily (e.g. during surgery or dental procedures, because of a serious injury, during childbirth, or due to heavy periods). However, with all forms of VWD, there can be bleeding problems.

There are three main types of VWD, based on the specific problem with the VWF protein. Bleeding symptoms can differ within each type. It is important to know which type of VWD a person has so that the right treatment is given.

If you suspect you might have VWD, visit www.VWDtest.com and complete the simple online bleeding questionnaire. 

Hematology treatment

FACTOR VIII REPLACEMENT is The Standard of Care for Hemophilia A:

• On-demand treatment is used to stop bleeds when they occur;

• Prophylactic therapy (two or three times per week) is used to prevent bleeding episodes;

• An active life can be maintained with proper treatment.