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Factor VII deficiency

Proconvertin deficiency; Extrinsic factor deficiency; Serum prothrombin conversion accelerator deficiency; Alexander disease

Factor VII (seven) deficiency is a disorder caused by a lack of a protein called factor VII in the blood. It leads to problems with blood clotting (coagulation).

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Blood clot formation
Blood clots

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Causes

When you bleed, a series of reactions take place in the body that helps blood clots form. This process is called the coagulation cascade. It involves special proteins called coagulation, or clotting factors. You may have a higher chance of excess bleeding if one or more of these factors are missing or are not functioning as they should.

Factor VII is one such coagulation factor. Factor VII deficiency runs in families (inherited) and is very rare. Both parents must have the gene to pass the disorder on to their children. A family history of a bleeding disorder can be a risk factor.

Factor VII deficiency can also be due to another condition or use of certain medicines. This is called acquired factor VII deficiency. It can be caused by:

Symptoms

Symptoms may include any of the following:

Exams and Tests

Tests that may be done include:

Treatment

Bleeding can be controlled by getting intravenous (IV) infusions of normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII.

You will need frequent treatment during bleeding episodes because factor VII does not last for long inside the body. A form of factor VII called NovoSeven can also be used.

If you have factor VII deficiency due to a lack of vitamin K, you can take this vitamin by mouth, through injections under the skin, or through a vein (intravenously).

If you have this bleeding disorder, be sure to:

Support Groups

These resources can provide more information on Factor VII deficiency:

Outlook (Prognosis)

You can expect a good outcome with proper treatment.

Inherited factor VII deficiency is a lifelong condition.

The outlook for acquired factor VII deficiency depends on the cause. If it is caused by liver disease, the outcome depends on how well your liver disease can be treated. Taking vitamin K supplements will treat vitamin K deficiency.

Possible Complications

Complications may include:

When to Contact a Medical Professional

Get emergency treatment right away if you have severe, unexplained bleeding.

Prevention

There is no known prevention for inherited factor VII deficiency. When a lack of vitamin K is the cause, using vitamin K can help.

Related Information

Central nervous system
Stroke

References

Gailani D, Wheeler AP, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 137.

Hall JE. Hemostasis and blood coagulation. In Hall JE, ed. Guyton and Hall Textbook of Medical Physiology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 37.

Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 174.

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Review Date: 1/29/2019  

Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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