Activated protein C resistance and factor V Leiden in patients with hemolysis, elevated liver enzymes, low platelets syndrome.
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).


CONCLUSIONS
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.
Department of Gynecology and Obstetrics, University of Göttingen, Germany. haugust@med.uni-goettingen.de
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D000328:Adult; D005165:Factor V / Q000032:analysis* / Q000235:genetics; D005260:Female; D017359:HELLP Syndrome / Q000209:etiology*; D006579:Heterozygote; D006801:Humans; D008875:Middle Aged; D011247:Pregnancy; D011486:Protein C / Q000032:analysis* / Q000235:genetics; D012307:Risk Factors
D011486:Protein C; D005165:Factor V