Mortality and inherited thrombophilia: results from the European Prospective Cohort on Thrombophilia

Abstract

To cite this article: Pabinger I, Vossen CY, Lang J, Conard J, García-Dabrio MC, Miesbach W, Legnani C, Svensson P, Kaider A, Rosendaal FR.Mortality and inherited thrombophilia: results from the European Prospective Cohort on Thrombophilia. J Thromb Haemost 2012; 10: 217-22.Summary. Background: Data on the survival of individuals with hereditary thrombophilia are rare and only come from retrospective studies. Objective: The aim of the present study was to assess mortality in individuals with known thrombophilia with and without a history of thrombosis in comparison to a control group. Patients/methods: The European Prospective Cohort on Thrombophilia (EPCOT) study is a prospective, multi-center observational study performed to assess the risk of thrombosis in persons with inherited thrombophilia. In an extension of the present study, the vital status was assessed in 1240 individuals with thrombophilia (mean age 40.9 years, 59% women, 196 with antithrombin, 341 with protein C, 276 with protein S-deficiency, 330 with factor (F)V Leiden and 97 with combined defects, and 62% with a history of venous thrombosis [VT]) and 875 controls (mean age 42.5 years, 48% women, 7% with a history of VT). Results: Seventy-two individuals with thrombophilia and 45 controls died during follow-up. The risk of death, adjusted for gender, thrombosis history and center, was not associated with thrombophilia (hazard ratio [HR] thrombophilia individuals vs. controls: 1.09, 95% confidence interval [CI] 0.66-1.78). When individuals with thrombophilia were evaluated separately, a history of thrombosis was not associated with mortality: the risk of death after adjustment for gender, anticoagulation and center was HR 0.79 (95% CI, 0.41-1.54). Conclusions: No increased risk of death in individuals with thrombophilia, not even in those with a history of thrombosis, was observed.

Increased mortality in patients with the lupus anticoagulant: the Vienna Lupus Anticoagulant and Thrombosis Study (LATS)

“…In a follow-up study of the European Prospective Cohort of Thrombophilia (EPCOT), no increased mortality in comparison with a control population was observed, not even in persons with thrombotic manifestations. 23 As clearly shown by our data and others, 20 patients with APS differ from these observations and have a considerably increased risk of long-term mortality.…”

“…In studies in patients without APS and with primary manifestation of DVT, mortality after the acute phase was mainly increased due to cancer-associated deaths 21,22 and survival was not impaired, if thrombotic manifestations triggered by cancer or aPLA positivity were excluded. [23][24][25] This has also been confirmed in patients with hereditary thrombophilia with and without thrombosis. In a follow-up study of the European Prospective Cohort of Thrombophilia (EPCOT), no increased mortality in comparison with a control population was observed, not even in persons with thrombotic manifestations.…”

A Biochemical Marker of Preeclampsia - Hypertriglyceridemia - and the Impact of Secondary Prevention Therapy

“…The most important result of our study is the statistically significant extremely low rate of complications in the treatment groups. The complete absence of major maternal and fetal complications (no case of acute pancreatitis, eclampsia, maternal or fetal death, abruptio placentae, severe intrauterine groth restriction) is an important argument for continuing the studies in this field, in order to find the most appropriate onset and dosages of treatment [12,13].…”

Single Nucleotide Variants in the Protein C Pathway and Mortality in Dialysis Patients

“…We showed in the current study that factor V Leiden was associated with increased all-cause mortality in dialysis patients. Other studies did not find an increased all-cause mortality risk for factor V Leiden in the general population and in thrombophilic families [23], [24]. However, it could be the interaction between dialysis and factor V Leiden that could lead to an increased mortality, which is not present in the general population.…”