Elsevier

Obstetrics & Gynecology

Volume 92, Issue 3, September 1998, Pages 457-460
Obstetrics & Gynecology

Original Articles
Activated protein C resistance and Factor V Leiden in patients with hemolysis, elevated liver enzymes, low platelets syndrome

https://doi.org/10.1016/S0029-7844(98)00208-7Get rights and content

Abstract

Objective: Hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is characterized by a distinct activation of the coagulation system. A mutation of the gene coding for coagulation Factor V (Factor V Leiden) has been identified as the most frequent risk factor for thrombosis. To identify risk factors for HELLP syndrome, we determined coagulation parameters and the Factor V Leiden mutation in women who previously had developed HELLP syndrome.

Methods: Coagulation parameters (activated protein C resistance, antithrombin, protein C, protein S) were determined in 21 women 6 months to 9 years after they had developed HELLP syndrome in the third trimester. In addition, these women were analyzed for the presence of the Factor V Leiden mutation.

Results: Of these analyzed women, 33% (seven of 21) had an activated protein C resistance (activated protein C ratio less than 2.0). Another 38% of the women had subnormal activated protein C ratios (2.0–2.3). Only 57% of the women with an activated protein C resistance were identified as heterozygous carriers of the Factor V Leiden mutation (four of seven).

Conclusion: Women with HELLP syndrome have a higher incidence of Factor V Leiden mutations. This increased incidence does not, however, account fully for the increased frequency of activated protein C resistance in these patients.

Section snippets

Materials and methods

A total of 80 patients with HELLP syndrome were diagnosed and treated at the University of Göttingen Medical School between 1987 and 1996. Of these, 21 women could be identified 6 months to 9 years after parturition who still lived in the Göttingen area, were willing to participate in the study, and were not users of oral contraceptives at the time of the study. Because none of these selection criteria had a direct relationship to any of the experimental parameters of the study, this selection

Results

Activated protein C resistance determinations now are employed widely. Nevertheless, results are known to vary significantly and the analytic systems are not standardized. We consequently determined the frequency distribution of activated protein C ratios in randomly selected plasma sample donors in the Göttingen (central Germany) area. As shown in Figure 1, a subgroup of 90% of these values were normally distributed, varying around a mean of 2.65. There was no difference between male and

Discussion

Thrombin converts fibrinogen to fibrin and activates coagulation Factors V and VIII. At the same time, excessive coagulation is balanced by a number of anticoagulative mechanisms. These include antithrombin and protein C, which act as key regulators of the coagulation system.11 Protein C is activated on the binding of thrombin to the endothelial cell surface protein thrombomodulin. Activated protein C inhibits the coagulation cascade by cleaving Factor Va and Factor VIIIa, which leads to the

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Supported by a grant from the Deutsche Forschungsgemeinschaft Kr1571/1-2.

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