Factor VII deficiency

Answer 1

Definition

Factor VII deficiency is an inherited disorder in which a lack (deficiency) of plasma protein factor VII leads to abnormal bleeding.

Alternative Names

Extrinsic factor deficiency

Causes, incidence, and risk factors

This disorder occurs when the body does not have enough factor VII, an important blood clotting protein.

When you bleed, the body launches a series of reactions that help the blood clot. The process involves special proteins called coagulation factors. Factor VII is one of many coagulation factors. The final product of these reactions is the blood clot.

When certain coagulation factors are missing, your blood may not clot normally and you can bleed abnormally. Bleeding can vary from mild to severe in the same person over time. Bleeding may begin during infancy or childhood.

Most often a deficiency of factor VII is caused by:

• Severe liver disease
• Use of drugs that prevent clotting (anticoagulants such as warfarin or coumadin)
• Lack of 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. It is very rare to be born with factor VII deficiency that is due to 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)

Signs and tests

• Partial thromboplastin time (PTT)
• Plasma factor VII activity
• Prothrombin time (PT)

Treatment

Patients can control bleeding episodes by receiving normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII through a vein (intravenous). People need frequent treatment during bleeding episodes because factor VII does not last for long inside the body.

Women can better control menstrual bleeding with oral Birth Control (contraceptives). A form of factor VII called Novo-7 can also be used.

If a lack of vitamin K is causing the disorder, you can take vitamin K by mouth, through injections under the skin, or intravenously.

Support Groups

You can often help the stress of illness by joining a support group where members share common experiences and problems. See hemophilia - resources.

Expectations (prognosis)

The outcome can be good with proper treatment.

This is a life-long disorder if you get it from your parents. If it is caused by liver disease, the outcome depends on how well you control the liver problem.

Complications

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

Calling your health care provider

Go to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding.

Prevention

Genetic counseling may be helpful for disorders that start at birth (congenital). Taking vitamin K can help if you have a vitamin K deficiency.

References

Kessler C. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa:Saunders Elsevier;2007:chap 180.

Answer 2

Definition

Factor VII deficiency is an inherited disorder in which a lack (deficiency) of plasma protein factor VII leads to abnormal bleeding.

Alternative Names

Extrinsic factor deficiency

Causes, incidence, and risk factors

When you bleed, the body launches a series of reactions that help the blood clot. This is called the coagulation cascade. The process involves special proteins called coagulation or clotting factors. When one or more of these clotting factors are missing, there is usually a higher chance of bleeding.

This disorder occurs when the body does not have enough factor VII, an important blood clotting protein. Most often a deficiency of factor VII is caused by:

• Lack of 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 that is due to 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)

Signs and tests

• Partial thromboplastin time (PTT)
• Plasma factor VII activity
• Prothrombin time (PT)

Diagnosing a bleeding disorder is important so that the doctor can take extra care if you need surgery, and can test or warn other family members who might be affected.

Treatment

Patients can control bleeding episodes by receiving normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII through a vein (intravenous). People 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 a lack of vitamin K is causing the disorder, you can take vitamin K 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 where members share common experiences and problems. See hemophilia - resources.

Expectations (prognosis)

The outcome can be good with proper treatment.

This is a life-long disorder if you get it from your parents. If it is caused by liver disease, the outcome depends on how well your liver problem can be treated.

Complications

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

Calling your health care provider

Go to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding.

Prevention

Genetic counseling may be helpful for disorders that start at birth (congenital). Taking vitamin K can help if you have a vitamin K deficiency.

References

Gailani d, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier;2008:chap 127.

Kessler C. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa:Saunders Elsevier;2007:chap 180.

Reviewed By

Review Date: 02/28/2011

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.