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Thromb Haemost 2001; 86(03): 800-803
DOI: 10.1055/s-0037-1616134
Review Articles
Schattauer GmbH

Risk of Pregnancy-related Venous Thrombosis in Carriers of Severe Inherited Thrombophilia[*]

Ida Martinelli
1   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milan, Italy
,
Cristina Legnani
2   Department of Angiology and Marino Golinelli Thrombophilia Unit, Sant’Orsola Hospital, Bologna, Italy
,
Paolo Bucciarelli
1   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milan, Italy
,
Elvira Grandone
3   Atherosclerosis and Thrombosis Unit, IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG), Italy
,
Valerio De Stefano
4   Department of Hematology, Catholic University, Rome, Italy
,
Pier Mannuccio Mannucci
1   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milan, Italy
› Author Affiliations
Further Information

Publication History

Received 12 March 2001

Accepted after revision 23 April 2001

Publication Date:
14 December 2017 (online)

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Summary

Homozygous carriers of factor V Leiden have an approximately 80-fold increased risk of venous thrombosis. Also double heterozygous carriers of both the factor V Leiden and the prothrombin gene mutations are at high thrombotic risk. The magnitude of the risk of venous thrombosis in pregnant women with the two severe thrombophilic conditions has not been estimated so far. We performed a multicenter retrospective family study in women with homozygous factor V Leiden, double heterozygous factor V Leiden and the prothrombin gene mutation, and women with normal coagulation. Only relatives of index patients with thrombosis formed the study cohort. Fifteen homozygous and 39 double heterozygous women were compared to 182 women with normal coagulation. Venous thrombosis occurred in 3 of 19, 2 of 50 and 1 of 221 pregnancies, respectively. One thrombotic episode occurred in the third trimester, the remaining 5 in the postpartum. The prevalence of venous thrombosis was 15.8% (95% CI 3.4-39.6) for homozygotes, 4.0% (95% CI 0.5-13.7) for double heterozygotes and 0.5% for women with normal coagulation. The relative risk of pregnancy-related venous thrombosis was 41.3 (95% CI 4.1-419.7) for homozygous and 9.2 (95% CI 0.8-103.2) for double heterozygous carriers. In conclusion, homozygous carriers of factor V Leiden and, to a lesser extent, double heterozygous carriers of factor V Leiden and of the prothrombin mutation have an increased risk of venous thrombosis during pregnancy, particularly high during the postpartum period. On the basis of these findings we recommend that these women receive anticoagulant prophylaxis at least in the postpartum, that should perhaps be extended to the whole pregnancy in homozygous carriers.

Keywords

Coagulation - factor V Leiden - prothrombin mutation - pregnancy - puerperium

* This work has been done within the frame of activity of the GIRTE (Italian Research Group on Inherited Thrombophilia).


 

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