Inherited thrombophilic states

Citation
Ak. Rao et al., Inherited thrombophilic states, SEM THROMB, 24, 1998, pp. 3-12
Citations number
83
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
SEMINARS IN THROMBOSIS AND HEMOSTASIS
ISSN journal
00946176 → ACNP
Volume
24
Year of publication
1998
Supplement
1
Pages
3 - 12
Database
ISI
SICI code
0094-6176(1998)24:<3:ITS>2.0.ZU;2-6
Abstract
Thrombophilic states are a group of conditions associated with increased pr edisposition to thromboembolic events. The well-recognized inherited thromb ophilic states include resistance to activated protein C (APC) (Factor V Le iden) and deficiencies of plasma antithrombin, protein C, and protein S, Th ese entities are aberrations in the natural anticoagulant systems that exis t in plasma and at the endothelial cell level. Other causes of inherited th rombophilia include hyperhomocysteinemia, abnormalities in the proteins of the fibrinolytic system, dysfibrinogenemias, deficiency of heparin cofactor II, abnormal thrombomodulin, and the recently described variation in the p rothrombin gene. Most of the inherited abnormalities recognized to date are associated with venous thromboembolism (VTE) rather than arterial thrombos is. Approximately half of VTE episodes in patients with inherited thromboph ilias occur in relation to events that are generally recognized as predispo sing states, such as surgery, pregnancy, and immobilization, In this review , the risks of VTE associated with inherited risk factors are discussed, an d guidelines for the diagnosis and management are presented.