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Measurement of basal levels of factor VIIa in hemophilia A and B patients
[see comments]
P Wildgoose, Y Nemerson, LL Hansen, FE Nielsen, S Glazer and U Hedner
Novo Nordisk A/S, Biopharmaceuticals Division, Gentofte, Denmark.
Previous results, presented in abstract form, indicate that replacement of
thromboplastin with a mixture of phospholipid and truncated soluble tissue
factor apoprotein results in a coagulation assay that can directly measure
plasma factor VIIa levels without interference from zymogen factor VII
(Atherosclerosis Thromb 11:1544a, 1991 [abstr]). We have exploited the
specificity and sensitivity of such a factor VIIa specific coagulation
assay to directly assess the in vivo relationship of factor VIII and factor
IX on the production of factor VIIa levels under nonthrombotic and
nonstimulatory conditions. Normal individuals (n = 20) were found to
possess an average circulating factor VIIa level corresponding to 4.34 +/-
1.57 ng/mL, or approximately 1% of their total factor VII antigen. Severe
factor VIII deficient patients (n = 13) possessed a slightly lower but
statistically significant (P less than .01) decrease in their basal factor
VIIa levels (2.69 +/- 1.52 ng/mL), corresponding to approximately 60% of
that observed in normal individuals. On the other hand, severe factor IX
deficient patients (n = 7) were found to possess even lower levels of
factor VIIa corresponding to 0.33 +/- 0.15 ng/mL, or less than 10% of that
observed in normal individuals. Measurement of total factor VII antigen
levels shows that the variation in basal factor VIIa levels stems from
differences in the degree of factor VII activation as opposed to
differences in factor VII antigen levels. Our present data are consistent
with the hypothesis that factor IXa is the principal in vivo activator of
factor VII under basal conditions.
Volume 80,
Issue 1,
pp. 25-28,
07/01/1992
Copyright © 1992 by The American Society of Hematology
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