Factor VII: More info
Shears: As already mentionned, a link for a UptoDate article would do no good unless you subscribe. Sorry.
"Also, how common is herditary Factor VII deficiency. Can it be acquired, and how common is that?"
I did some digging on that question.
Below is some information ---actually more info than one should probably ever need---on Factor VII. It also addresses the interesting question posed here about whether there are ethnic or geographic variations in deficiency prevalence that might help make marketing and distribution more cost-effective.
This also is from UpToDate, so no link provided.
The X-linked inherited coagulation disorders, hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), together with von Willebrand disease, comprise 95 to 97 percent of all the inherited deficiencies of coagulation factors [1,2]. The remaining defects are rare and generally transmitted as autosomal recessive disorders, with prevalences ranging from approximately 1 in 500,000 to 1 in 2 million [3-5]. Higher prevalences are found in countries with large Jewish communities (factor XI deficiency) and regions (eg, Middle Eastern countries, Southern India) where consanguineous marriages are relatively common [6].
NB: This does not really address directly the geographic or ethnic predisposition specifically for Factor VII deficiency. The numbers are just too small.
Factor VII deficiency — Factor VII deficiency has an estimated incidence of 1:500,000. The factor VII gene maps on chromosome 13 close to that encoding factor X. More than two thirds of factor VII mutations have been missense mutations; the remaining ones are null mutations that decrease or abolish the expression of factor VII [4,47-49]. In general, patients with mild to moderate clinical phenotypes are homozygous or compound heterozygous for missense mutations, whereas more severe phenotypes are associated with deletions, insertions, splicing, and promoter mutations. However, some missense mutations are also associated with a severe phenotype [50].
The most common clinical symptoms, other than excessive bleeding after invasive procedures, include mucosal tract, joint, and muscle bleeding. As noted above, factor VII deficiency presents with a wide spectrum of clinical severity, correlating poorly with factor VII levels [30-32]; some patients with undetectable levels are asymptomatic [21,26]; there is no explanation for this observation. Intracranial bleeding, reported to be frequent and severe after birth in one series of factor VII-deficient patients [33] was rare in the Iranian, Italian, and American cohorts [21,26].
(This refers to the three main registries for Factor VII deficient patients which allowed the genetics to be worked out.)
Here is some interesting info on treatment:
Treatment — The very short half-life of factor VII (4 to 6 hours) makes it difficult to use FFP without causing volume overload. Recombinant human activated factor VII is licensed for use in factor VII deficiency in both Europe and the United States [4,51,52]....
The recommended dosage of recombinant human factor VIIa, 15 to 30 microg/kg IV, repeated every 12 hours, according to the given clinical situation, is able to keep factor VII levels above 15 to 20 percent.
Two commercial manufacturers produce virus-inactivated factor VII concentrates, used successfully in small series of patients [53,54]. The recommended starting dose is 30 to 40 U/kg IV, repeated every 12 hours as necessary.
I haven't heard of the other product, only NovoSeven.
Finally, regarding the question on causes for ACQUIRED factor VII deficiency, these are almost always going to be in DIC (which consumes all types of circulating clotting factors) or severe liver disease (in which the body loses the ability to produce clotting factors.) In both of these disease states, the defect is not limited to Factor VII and I think specific Factor VII replacement would probably be inappropriate.
As I said, this is more than I ever wanted to know about Factor VII. But thanks for the opportunity to do some digging.
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