Parahemophilia: New Insights into Factor V Deficiency

Nabil Thalji; Rodney M. Camire

doi:10.1055/s-0033-1349224

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2013; 39(06): 607-612
DOI: 10.1055/s-0033-1349224

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Parahemophilia: New Insights into Factor V Deficiency

Nabil Thalji

¹Division of Hematology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, The University of Pennsylvania, Philadelphia, Pennsylvania

,

Rodney M. Camire

¹Division of Hematology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, The University of Pennsylvania, Philadelphia, Pennsylvania

› Author Affiliations

Abstract

Blood coagulation factor V (FV) plays a pivotal role in blood coagulation. It is found in both plasma and in platelets and has a profound impact on thrombin generation. Deficiency of this clotting factor due to inherited or acquired conditions results in a broad spectrum of bleeding symptoms. Surprisingly however, some patients with undetectable levels of FV experience relatively mild bleeding. The aim of this review is to highlight this rare coagulation factor disorder and touch upon its clinical manifestations, diagnosis, and treatment. Furthermore, recent advances that shed new light on the importance of platelet FV and other modifiers which influence bleeding tendencies in severe FV deficiency will be discussed.

Keywords

factor V - factor V deficiency - hemostasis - bleeding phenotype

Full Text

References

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