Factor VII deficiency is a blood
clotting disorder that causes excessive or prolonged bleeding after an injury
or surgery. With factor VII deficiency, your body either doesn’t produce enough
factor VII, or something is interfering with your factor VII, often another
Factor VII is a protein produced
in the liver that plays an important role in helping your blood to clot. It’s
one of about 20 clotting factors involved in the complex process of blood
clotting. To understand factor VII deficiency, it helps to understand the role
factor VII plays in normal blood clotting.
Role Does Factor VII Play in Normal Blood Clotting?
The normal blood clotting process
occurs in four stages:
When a blood vessel is cut, the
damaged blood vessel immediately constricts to slow blood loss. Then, the
injured blood vessel releases a protein called tissue factor into the bloodstream.
The release of tissue factor acts like an SOS call, signaling blood platelets
and other clotting factors to report to the scene of the injury.
2. Formation of a Platelet Plug
Platelets in the bloodstream are
the first to arrive at the injury site. They attach themselves to the damaged
tissue, and to each other, forming a temporary, soft plug in the wound. This
process is known as primary hemostasis.
3. Formation of a Fibrin Plug
Once the temporary plug is in
place, the blood clotting factors go through a complex chain reaction to
release fibrin, a tough, stringy protein. Fibrin wraps itself in and around the
soft clot until it becomes a tough, insoluble fibrin clot. This new clot seals
the broken blood vessel, and creates a protective covering for new tissue
4. Wound Healing and Destruction
of the Fibrin Plug
After a few days, the fibrin clot
starts to shrink, pulling the edges of the wound together to help new tissue
grow over the wound. As the tissue is rebuilt, the fibrin clot dissolves and is
If factor VII does not function
properly, or there is too little of it, the stronger fibrin clot cannot form
Causes Factor VII Deficiency?
Factor VII deficiency may be
either inherited or acquired. The inherited version is quite rare. Fewer than
200 documented cases have been reported. Both of your parents must carry the
gene in order for you to be affected.
Acquired factor VII deficiency,
in contrast, occurs after birth. It can occur as a result of medications or
diseases that interfere with your factor VII. Drugs that can impair or reduce
factor VII function include:
thinners, such as warfarin
- some cancer
drugs, such as interleukin-2 therapy
globulin therapy used to treat aplastic anemia
Diseases and medical conditions
that can interfere with factor VII include:
Are the Symptoms of Factor VII Deficiency?
Symptoms vary from mild to
severe, depending on your levels of usable factor VII. Mild symptoms might
- bruising and
soft tissue bleeding
bleeding time from wounds or dental extractions
- bleeding in
In more severe cases, symptoms
of cartilage in joints from bleeding episodes
- bleeding in
the intestines, stomach, muscles, or head
bleeding after childbirth
Is Factor VII Deficiency Diagnosed?
Diagnosis is based on your
medical history, any family history of bleeding problems, and lab tests.
tests for factor VII deficiency
assays to identify missing or poorly performing factors
- factor VII
assay to measure how much factor VII you have, and how well it works
time (PT) to measure the functioning of factors I, II, V, VII, and X
prothrombin time (PTT) to measure the functioning of factors VIII, IX, XI, XII,
and von Willebrand factors
tests to determine if your immune system is attacking your clotting factors
Is Factor VII Deficiency Treated?
Treatment of factor VII
deficiency focuses on:
- controlling bleeding
- resolving underlying conditions
- precautionary treatment before surgery or
During bleeding episodes, you may
be given infusions of blood clotting factors to boost your clotting ability.
Clotting agents commonly used include human prothrombin complex,
cryoprecipitate, fresh frozen plasma, or recombinant human factor VIIa
Treatment of Underlying Conditions
Once bleeding is under control,
conditions that impair factor VII production or functioning, such as
medications or diseases, must be addressed.
Precautionary Treatment Before Surgery
If you’re planning surgery, your
doctor may prescribe drugs to minimize your risk of excessive bleeding.
Desmopressin nasal spray is often prescribed to release all available stores of
factor VII before minor surgery. For more serious surgeries, your doctor may
prescribe infusions of clotting factor.
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.