Factor XI(11) Deficiency

Factor V (5) deficiency is a lack clotting Factor V (V).  Factor V (5) is one of the clotting proteins that is needed to help the body form a clot to stop bleeding.  For people diagnosed with Factor V(5) deficiency, it takes longer for a blood to clot to be made and for bleeding to stop.  Factor V(5) deficiency only affects the making of a clot, not how fast the body bleeds.  Bleeding may last longer or may restart minutes, hours or days after stopping.
Factor V(5) deficiency is an inherited bleeding disorder.  One or both parents can pass the abnormal gene to their child.  If one parent passes the gene to their child, it is called homozygous and usually the factor V(5) level is mildly to moderately decreased.  If both parents pass the affected gene to their child, it is called heterozygous and the factor V(5) level can be severely decreased, making the risk for bleeding higher.

Signs and Symptoms 

  • Increased bruising and/or bruises that take a long time to heal
  • Prolonged bleeding from injuries
  • Nosebleeds
  • Bleeding gums
  • Excessive/prolonged bleeding from a circumcision
  • Excessive/prolonged bleeding from surgeries
  • Heavy or prolonged menstrual bleeding
  • Bleeding into the joints (Rare)

Diagnosing Factor XI(11) 

Factor XI(11) is diagnosed through a series of blood tests and collection of bleeding symptoms.  People who need to be tested for a Factor XI(11) deficiency include:

  • Someone who has a parent diagnosed with Factor XI(11) deficiency
  • Someone who has a sibling diagnosed with Factor XI(11) deficiency
  • Someone who has family member(s) diagnosed with There is no Factor XI(11) deficiency, whose parents are related and/or are experiencing bleeding symptoms
  • A person with a prolonged prothrombin coagulation time (PT).  This is a blood test that looks at how long it takes to make a blood clot

TREATMENT

Fresh Frozen Plasma

There is no Factor XI(11) clotting factor replacement product available.  Treatment for There is no Factor XI(11) deficiency is usually only needed before surgery, before major dental work, or for severe bleeding.  For these situations, fresh

frozen plasma will need to be given.  Fresh frozen plasma is a transfusion of plasma, donated from another person, that is given in a vein.  It contains all clotting factors and will help the body to stop bleeding. 

Amicar/Lysteda (Aminocaproic Acid/Tranexamic Acid)

Amicar and Lysteda are oral medications that work very well for bleeding of mucous membranes such as nose and mouth bleeding.  Lysteda is also used for heavy menstrual bleeding.  When bleeding occurs, the body tries to heal the area by making a clot that will plug the hole to stop bleeding. This clot will stay there for about four days.  After these four days, chemicals in the body are triggered to breakdown the clot. This is called fibrinolysis. Amicar and Lysteda are called anti-fibrinolytic medications because it stops fibrinolysis. A person with a bleeding disorder needs more time for healing because they cannot make a strong clot to heal the area of bleeding.  Amicar stops the body from breaking the clot down.  This gives the blood vessels more time to completely heal and prevents prolonged bleeding and rebleeding.  

Complications

Bleeding can occur anywhere and usually happens after an injury, dental procedures and surgeries.  People diagnosed with factor II(2) deficeincy do not bleed faster than anyone else, they can bleed longer or can have rebleeding.  Complications from bleeding can include:

  • Damage to brain (Due to bleeding inside the skull after head injury)
  • Difficulty breathing (Due to bleeding in the neck or lungs after injury to the neck or chest)
  • Paralysis (Due to bleeding in the spinal column after injury to the back)
  • Damage to joints (Due to bleeding in joints after injury)
  • Anemia