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

• Published in: Obstetrics and gynecology (New York. 1953) Vol. 92; no. 3; pp. 457 - 460
• Main Authors: Krauss, Thomas, Augustin, Hellmut G, Osmers, Rüdiger, Meden, Harald, Unterhalt, Michael, Kuhn, Walther
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.09.1998; The American College of Obstetricians and Gynecologists; Elsevier Science
• Subjects: Adult; Biological and medical sciences; Diseases of mother, fetus and pregnancy; Factor V - analysis; Factor V - genetics; Female; Gynecology. Andrology. Obstetrics; HELLP Syndrome - etiology; Heterozygote; Humans; Medical sciences; Middle Aged; Pregnancy; Pregnancy. Fetus. Placenta; Protein C - analysis; Protein C - genetics; Risk Factors; Human; Statistical analysis; Pregnancy disorders; HELLP syndrome; Hepatic disease; Protein C; Hemopathy; Incidence; Factor V; Resistance; Gene; Preeclampsia; Risk factor; Digestive diseases; Female; Mutation
• ISSN: 0029-7844; 1873-233X
• DOI: 10.1016/S0029-7844(98)00208-7

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.