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Hemophilia C also known as Factor XI Deficiency is inherited in an autosomal dominant fashion. This means it affects men and women equally since it is not carried on the X chromosome and a single gene for the condition is enough to cause the disorder. There can be quite a bit of variability in severity with this deficiency. Individuals  are not likely to bleed spontaneously, and hemorrhaging normally occurs after trauma or surgery. Joint bleeds are uncommon. Patients are more prone to bruising, nosebleeds, or blood in the urine. Woman may experience menorrhagia and prolonged bleeding after childbirth.

Hemophilia C

Also known as Alexander's disease, and serum pro thrombin version accelerator (SPCA) deficiency, it is an inherited autosomal blood disorder similar to hemophilia. It affects men and women equally because unlike hemophilia it is not carried on the X chromosome. Factor VII deficiency is usually severe. People with severe factor VII are prone to joint bleeds.

Factor VII Deficiency

Diagnosis

Diagnosis is made through bleeding time tests, platelet function tests, prothrombin time (PT) and activated partial thromboplastin time (aPTT) tests. A specific Factor XI assay is extremely useful in ruling out combined deficiencies.

Treatment

While there are two factor XI concentrates produced in Europe, in the United States there are no factor XI concentrates available and fresh-frozen plasma is normally used for treatment. Since Factor XI is not concentrated in fresh frozen plasma, considerable amounts of plasma may be required to maintain the factor level.

Diagnosis

Diagnosis is made through

1) activated partial thromboplastin time (aPTT) test,

2)pro thrombin time (PT) test and

3) thrombin time (TT) test

 Diagnosis can be confirmed with a factor VII assay.

Treatment

Like Hemophilia's A & B, a factor concentrate is available for intravenous infusions, replacing the Factor VII.

Pro thrombin complex concentrates (PCCs) can be used to treat Factor VII deficiency, but factor content can also vary between product lots produced, even by the same manufacturer.

Factor VII deficiency can also be treated with fresh frozen plasma (FFP).

What Are the Symptoms of Factor VII Deficiency?

Symptoms vary from mild to severe, depending on your levels of usable factor VII.

Mild symptoms might include:

bruising and soft tissue bleeding

longer bleeding time from wounds or dental extractions

bleeding in joints

nosebleeds

bleeding gums

heavy menstrual periods      


In more severe cases, symptoms can include:

destruction of cartilage in joints from bleeding episodes

bleeding in the intestines, stomach, muscles, or head

excessive bleeding after childbirth

What Is the Long-Term Outlook?

If you have the acquired form of factor VII deficiency, it’s probably caused by either medications or an underlying condition. Your long-term outlook depends on fixing the underlying problems. If you have the more severe inherited form of factor VII deficiency, you will need to work closely with your doctor and your local hemophilia center to manage bleeding risks.

Living with Hemophilia C

Although patients with hemophilia C may experience milder symptoms than those living with other types of hemophilia, this disorder should not be overlooked. People living with hemophilia C require effective treatment to achieve optimal outcomes after surgeries or injuries. For this reason, it is critical that patients inform their physicians of their diagnosis prior to any planned procedures

Complications of Hemophilia C

The complications of hemophilia C are those primarily associated with the use of fresh frozen plasma. While today’s blood products are much safer than those of the past, transmission of hepatitis A, hepatitis C, and newly discovered blood-borne diseases remain a risk for people treated with plasma-derived products.

Immunization against hepatitis A and B is recommended for all individuals with hemophilia.

For more specific and factual information on Hemophilia's C &Factor VII, including cause, symptoms, diagnosis, treatments, and more visit: