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(Arteriosclerosis, Thrombosis, and Vascular Biology. 2005;25:1992.)
© 2005 American Heart Association, Inc.

Thrombosis

Recurrence Rate After a First Venous Thrombosis in Patients With Familial Thrombophilia

Carla Y. Vossen; Isobel D. Walker; Peter Svensson; Juan C. Souto; Inge Scharrer; F. Eric Preston; Gualtiero Palareti; Ingrid Pabinger; Felix J.M. van der Meer; Mike Makris; Jordi Fontcuberta; Jacqueline Conard; Frits R. Rosendaal

From the Department of Clinical Epidemiology (C.Y.V., F.R.R.), Leiden University Medical Center, Leiden, The Netherlands; the Department of Haematology (I.D.W.), Glasgow Royal Infirmary, Glasgow, UK; the Department for Coagulation Disorders (P.S.), University Hospital, Malmö, Sweden; the Department of Haematology (J.C.S., J.F.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; the Department of Internal Medicine (I.S.), University Hospital, Frankfurt/Main, Germany; the Department of Haematology (F.E.P., M.M.), Royal Hallamshire Hospital, Sheffield, UK; the Department of Angiology and Blood Coagulation (G.P.), University Hospital S. Orsola, Bologna, Italy; the Department of Haematology and Haemostaseology (I.P.), University Hospital Vienna, Vienna, Austria; the Department of Haematology (F.J.M.v.d.M., F.R.R.), Leiden University Medical Center, Leiden, The Netherlands; and the Department of Biological Haematology (J.C.), Hôtel-Dieu Hospital, Paris, France.

Correspondence to Prof F.R. Rosendaal, MD, Department of Clinical Epidemiology, Leiden University Medical Center, PO box 9600, 2300 RC Leiden, The Netherlands. E-mail F.R.Rosendaal{at}lumc.nl

Objective— Few comprehensive data are available on the recurrence rate of venous thrombosis in carriers of thrombophilic defects from thrombophilic families. We prospectively determined the recurrence rate after a first venous thrombotic event in patients with familial thrombophilia attributable to factor V Leiden or deficiencies of protein C, S, or antithrombin.

Methods and Results— Data were gathered during follow-up on the occurrence of risk situations, anticoagulation treatment, and events (eg, venous thrombosis, hemorrhage). Over a mean follow-up period of 5.6 years, 44 of the 180 patients with familial thrombophilia who did not use long-term anticoagulation experienced a recurrent venous thromboembolic event (5.0%/year; 95% CI 3.6 to 6.7) compared with 7 of the 124 patients on long-term anticoagulation (1.1%/year; 95% CI 0.4 to 2.2). Spontaneous events occurred less often in patients on long-term anticoagulation (57%) than in patients without long-term anticoagulation (75%). The highest recurrence rate was found among men with a deficiency in natural anticoagulants or multiple defects and women with antithrombin deficiency. Although long-term anticoagulation treatment decreased the incidence of recurrent events by 80%, it also resulted in a risk of major hemorrhage of 0.8% per year.

Conclusions— Extra care after a first event is required for men with a deficiency in natural anticoagulants or multiple defects and women with antithrombin deficiency.

We prospectively determined the recurrence rate in patients with familial thrombophilia. The overall incidence of a second venous thromboembolic event was 5.0%/year. Extra care after a first event is required for men with a deficiency in natural anticoagulants or multiple defects and women with antithrombin deficiency.

Key Words: familial thrombophilia • incidence • prospective follow-up • recurrence • venous thrombosis

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