Do prothrombotic factors influence clinical phenotype of severe haemophilia? A review of the literature

Karin van Dijk; Johanna G. van der Bom; Kathelijn Fischer; Diederick E. Grobbee; H. Marijke van den Berg

doi:10.1160/TH04-02-0112

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2004; 92(02): 305-310
DOI: 10.1160/TH04-02-0112

Review Article

Schattauer GmbH

Do prothrombotic factors influence clinical phenotype of severe haemophilia? A review of the literature

Karin van Dijk

¹Van Creveldkliniek, Dutch National Hemophilia Treatment Center, UMC Utrecht,The Netherlands

²Julius Center for Health Sciences and Primary Care, UMC Utrecht,The Netherlands

,

Johanna G. van der Bom

,

Kathelijn Fischer

¹Van Creveldkliniek, Dutch National Hemophilia Treatment Center, UMC Utrecht,The Netherlands

⁴Wilhelmina Children’s Hospital, UMC Utrecht,The Netherlands

,

Diederick E. Grobbee

²Julius Center for Health Sciences and Primary Care, UMC Utrecht,The Netherlands

,

H. Marijke van den Berg

¹Van Creveldkliniek, Dutch National Hemophilia Treatment Center, UMC Utrecht,The Netherlands

⁴Wilhelmina Children’s Hospital, UMC Utrecht,The Netherlands

› Author Affiliations

Abstract

Summary

There is considerable variability in bleeding patterns of severe haemophilia (<1% factor VIII). Knowledge of the contribution of thrombophilic factors in these patterns may improve individually tailored treatment strategies. We reviewed the literature regarding the relation between prothrombotic factors and clinical phenotype of severe haemophilia. Medline and EMBASE were searched for relevant articles. 9369 articles published between 1963 and September 2003 were screened and seven relevant papers were retrieved. Each of these reported on a different combination of thrombophilic factors. Presence of the factor V Leiden mutation appears to decrease the severity of severe haemophilia most consistently. Findings on other thrombophilic factors were inconclusive. There is a clear need for additional research on potential determinants of phenotypes of severe haemophilia before such knowledge can be translated into individual care for severe haemophilia patients with confidence.

Keywords

Severe haemophilia - phenotype - prothrombotic factors

Full Text

References

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