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Hemostatic enzyme generation in the blood of patients with hereditary
protein C deficiency
KA Bauer, AW Broekmans, RM Bertina, J Conard, MH Horellou, MM Samama and RD Rosenberg
Charles A. Dana Research Institute, Boston, MA.
The presence of hereditary protein C deficiency has been shown to
predispose patients to the development of venous thrombosis. We used
radioimmunoassays for the protein C activation peptide (PCP) and the
prothrombin fragment F1 + 2 to quantitate the extent of in vivo activation
of protein C by thrombin-thrombomodulin and prothrombin by factor Xa,
respectively, in the blood of individuals with this clinical disorder. A
total of 46 protein C deficient subjects from 18 kindreds were studied. In
23 nonanticoagulated patients with an isolated deficiency of protein C, the
mean level of PCP was substantially reduced while the mean concentration of
F1 + 2 was significantly elevated as compared with normal controls (1.10
pmol/L v 1.78 pmol/L, P less than .0005 and 2.54 nmol/L v 1.51 nmol/L, P
less than .0005, respectively). The metabolic behavior of 131I-F1 + 2 was
found to be similar in protein C deficient patients and normal individuals.
However, we were unable to establish a significant correlation between
decreased PCP levels and increased F1 + 2 measurements in these 23
patients. This study demonstrates that heterozygous protein C deficient
individuals with equivalent plasma levels of the zymogen may have markedly
different biochemical profiles when assay techniques are used that
quantitate the in vivo activity of the coagulation system. Six individuals
from three pedigrees were identified as having combined deficiencies of
protein C and either antithrombin III or protein S; the genetic basis for
the combined deficiency state was determined in two of the kindreds.
Finally we observed that hemostatic system activity as measured by the PCP
and F1 + 2 assays is markedly suppressed in protein C deficient patients
who are chronically anticoagulated with coumarin derivatives.
Volume 71,
Issue 5,
pp. 1418-1426,
05/01/1988
Copyright © 1988 by The American Society of Hematology
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