An hereditary deficiency in Factor VII can result in a lifelong bleeding disorder.


Inheritance: autosomal recessive, with consanguinity relatively common in affected families.


Clinical findings:

(1) An affected patient may show a variable bleeding tendency that depends on the level of Factor VII present. A patient with a major factor deficiency may experience excessive bleeding resembling hemophilia.

(2) Bleeding may occur after trauma or surgery or dental extraction.

(3) Spontaneous bleeding can occur in homozygous patients with severe factor deficiency.

(4) Other family members may be affected, with both genders affected.


Bleeding manifestations:

(1) easy bruisability

(2) soft tissue hemorrhages

(3) epistaxis

(4) hemarthroses with arthropathy

(5) intracranial hemorrhage, especially in neonates after vaginal delivery

(6) menorrhagia, metrorrhagia and postpartum bleeding in women


Some kindreds have thromboembolic events, which can be linked to a protein C deficiency or other hypercoagulable state.


Laboratory findings (assuming no factor replacement):

(1) prolonged PT

(2) normal aPTT and thrombin time

(3) decreased Factor VII antigen and/or activity by specific assays


Differential diagnosis of a hereditary deficiency:

(1) other hereditary coagulation factor deficiency

(2) acquired factor VII deficiency in vitamin K deficiency or after warfarin therapy

(3) liver disease

(4) factor VII inhibitor


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