Factor VII deficiency: Year of the Zebra 2026

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Introduction0:00–1:42

Factor 7 deficiency is a rare bleeding disorder in which the body, specifically the liver, doesn't produce enough functional factor 7.
During coagulation, a group of specialized proteins known as clotting factors activate one another in a rapid chain reaction called the coagulation cascade.
Each clotting factor passes the signal on to the next one, eventually activating thrombin, which converts fibrinogen into fibrin.
Then fibrin combines with itself many times, forming a mesh around the platelet plug that seals the injured vessel and creates a stable clot.
To prevent clots from forming unnecessarily, the coagulation cascade stays mostly inactive while blood vessels remain intact.
One important safeguard is tissue factor, a protein that's normally hidden within the vessel walls. When a blood vessel is damaged, tissue factor becomes exposed to the bloodstream.
As a result, circulating factor 7 binds to the exposed tissue factor, forming a complex that starts the coagulation cascade.
In factor 7 deficiency, this first step doesn't work as efficiently as it should, making it harder for the body to form stable clots and stop bleeding.
Most cases occur because of inherited mutations in the F7 gene, which codes for factor 7. The disorder usually follows an autosomal recessive inheritance pattern, meaning a person must inherit two altered copies of the gene, one from each parent, to develop the condition.
Rarely, factor 7 deficiency can be acquired later in life due to conditions like severe liver disease. Clinically, individuals with factor 7 deficiency have an increased risk of bleeding, though the severity varies widely.

Clinical Manifestations1:42–2:18

Newborns may present with bleeding from the umbilical stump or after procedures such as circumcision. Children and adults might notice frequent nosebleeds, easy bruising, or prolonged bleeding after minor injuries or dental work.
Some individuals might also experience heavy menstrual bleeding or develop bleeding into joints or muscles, similar to what occurs in hemophilia.
In rare but serious cases, bleeding can occur in the brain or gastrointestinal tract. Factor 7 deficiency is often suspected when routine blood tests show altered coagulation results.

Diagnosis2:18–2:57

Because factor 7 is involved in the first steps of the coagulation cascade, problems with this factor slow down how quickly clotting begins.
This is reflected in a blood test called prothrombin time, which measures how long it takes for clotting to start and is therefore prolonged.
On the flip side, another blood test called the activated partial thromboplastin time, which looks at different parts of the clotting process, is typically normal.
To confirm the diagnosis, laboratories measure the amount of factor 7 activity in the blood. Treatment of factor 7 deficiency is generally needed in cases of severe bleeding or before major surgery.

Treatment2:57–3:36

It usually involves intravenous or IV replacement therapy with recombinant activated factor 7, a laboratory-made version of the protein that restores clotting activity and helps the coagulation cascade move forward.
If that's not available, factor 7 can also be supplied through fresh frozen plasma. Or prothrombin complex concentrates which provide multiple clotting factors in addition to factor 7.
Anti-fibrinolytics, which help prevent clots from breaking down too quickly, may also be given to stop the bleeding. All right, as a quick recap, factor 7 deficiency is a bleeding disorder where the body lacks one of the clotting factors that starts the coagulation cascade.

Review3:36–4:01

This results in frequent nosebleeds, bruising, heavy menstrual bleeding, and more rarely, bleeding in internal organs, joints, or muscles.
Diagnosis is made through lab tests and treatment focuses on managing bleeding and recombinant factor 7 when necessary.