Anesthetic management of parturients with defects in coagulation factor V

Abstract

An inherited defect of coagulation factor V (FV) is a rare bleeding disorder with a prevalence of 1 in 1 000 000. 1 Asselta R. Tenchini M.L. Duga S. Inherited defects of coagulation factor V: the hemorrhagic side. J Thromb Haemost. 2006; 4: 26-34 Crossref PubMed Scopus (69) Google Scholar Defects can be classified as quantitative (type I) or qualitative (type II). Severe type I deficiency is defined as a level of FV antigen <15% of normal which may be associated with moderate to severe bleeding. 1 Asselta R. Tenchini M.L. Duga S. Inherited defects of coagulation factor V: the hemorrhagic side. J Thromb Haemost. 2006; 4: 26-34 Crossref PubMed Scopus (69) Google Scholar Although single cases and small series have been reported, life threatening bleeding seems rare; 1 Asselta R. Tenchini M.L. Duga S. Inherited defects of coagulation factor V: the hemorrhagic side. J Thromb Haemost. 2006; 4: 26-34 Crossref PubMed Scopus (69) Google Scholar , 2 Huang J.N. Koerper M.A. Factor V deficiency: a concise review. Haemophilia. 2008; 14: 1164-1169 Crossref PubMed Scopus (98) Google Scholar FV activity appears to have limited correlation with bleeding severity, even in the presence of very low FV antigen levels. 3 Lak M. Sharifian R. Peyvandi F. Mannucci P.M. Symptoms of inherited factor V deficiency in 35 Iranian patients. Br J Haematol. 1998; 103: 1067-1069 Crossref PubMed Scopus (104) Google Scholar Mild type I deficiency is characterized by FV plasma levels <60–65% with patients remaining asymptomatic. 1 Asselta R. Tenchini M.L. Duga S. Inherited defects of coagulation factor V: the hemorrhagic side. J Thromb Haemost. 2006; 4: 26-34 Crossref PubMed Scopus (69) Google Scholar , 4 Vellinga S. Steel E. Vangenechten I. Gadisseur A. Successful pregnancy in a patient with factor V deficiency: case report and review of the literature. Thromb Haemost. 2006; 95: 896-897 PubMed Google Scholar