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Factor VII deficiency

Definition

Factor VII deficiency is an inherited disorder. A lack of plasma protein factor VII leads to abnormal bleeding.

Alternative Names

Extrinsic factor deficiency

Causes

When you bleed, a series of reactions take place in the body that helps healthy blood clots form. This is called the coagulation cascade. The process involves special proteins called coagulation or clotting factors. You may have a higher chance of excess bleeding if one or more of these factors is missing. Factor VII deficiency occurs when the body does not have enough of a blood clotting protein called factor VII. Most often the lack of factor VII is caused by:

  • Low vitamin K due to long-term use of antibiotics, bile duct obstruction, or poor absorption of vitamin K from the intestines. Some babies are born with vitamin K deficiency.
  • Severe liver disease
  • Use of drugs that prevent clotting (anticoagulants such as warfarin or Coumadin)

It is very rare to be born with factor VII deficiency cause by the body's inability to make working factor VII.

Symptoms

  • Bleeding from mucus membranes
  • Bleeding into joints
  • Bleeding into muscles
  • Excessive bruising
  • Excessive menstrual bleeding
  • Nosebleeds (epistaxis)

Exams and Tests

Knowing that you have a bleeding disorder helps the doctor can take extra care if you need surgery. It also lets you alert also tell other family members who may have the same problem.

Treatment

You can control  bleeding episodes by receiving normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII through a vein (intravenous).

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.For a lack of vitamin K, you can take doses of this vitamin by mouth, through injections under the skin, or through a vein (intravenously).

Support Groups

You can often help the stress of illness by joining a support group [link to 01-2204] where members share common experiences and problems.

Outlook (Prognosis)

You can expect a good outcome with proper treatment. .

If the problem is  caused by liver disease, the outcome depends on how well your liver problem can be treated.

Possible Complications

  • Excessive bleeding (hemorrhage)
  • Stroke or other nervous system problems from central nervous system bleeding
  • Joint problems in severe cases when bleeding happens often

When to Contact a Medical Professional

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

Prevention

Genetic counseling may be helpful for disorders that start at birth. Taking vitamin K can help if you have a lack of this vitamin.

References

Ragni MV. Hemorrhagic Disorders: Coagulation Factor Deficiencies. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 177.

Gailani D, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 139.


Review Date: 3/3/2013
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.
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