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Hemophilia Specialty Pharmacy

A disorder affecting the blood’s ability to properly clot

Conditions

Hemophilia is an inherited disorder that causes abnormal bleeding. The bleeding occurs because part of the blood -- called plasma -- has too little of a protein that helps blood clot. Symptoms of hemophilia range from increased bleeding after trauma, injury, or surgery to sudden bleeding with no apparent cause. There are three types of hemophilia:

  • Hemophilia A results from too little of a plasma protein called factor VIII, which helps blood clot. The greater the deficiency, the more severe the symptoms.

  • Hemophilia B results from too little of a plasma protein called factor IX, which helps blood clot. As in hemophilia A, hemophilia B can be mild, moderate, or severe. The greater the deficiency, the more severe the symptoms.

  • Hemophilia C is a rare genetic disorder caused by missing or defective blot clotting protein called Factor XI. The disease was first recognized in 1953 in patients who experienced severe bleeding after dental extractions and to this day, it is still not very well-known.

  • Von Willebrand Disease (VWD) is a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein. VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process.

Symptoms of Hemophilia include excessive bruising, swollen painful joints, swollen tender muscles, excessive bleeding from gums/tongue/mouth following injury (seen particularly in infants and toddlers), severe bleeding after tooth extractions or other invasive dental procedures, and severe bleeding after injuries or operations.

Hemophilia can be treated by replacing missing blood clotting factors. This is called clotting factor replacement therapy. Clotting factors are replaced by injecting (infusing) a clotting factor concentrate into a vein. Infusions of clotting factors help blood to clot normally. Clotting factor replacement therapy can treat/help control bleeding episodes or prevent bleeding. It can prevent severe blood loss and complications from bleeding such as damage to muscle, joints, and organs.

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