Coagulation factor V inhibitors, a review of the case report literature

Abstract

Background: Coagulation factor V (FV) inhibitor is a rare disease and few reviews of relevant literature on FV inhibitor have been reported. Methods: A PubMed search identified 1065 reports on factor V inhibitors, in which 150 patients met the criteria for inclusion in this review. Result: One hundred fifty cases with prolonged clotting times and FV inhibitor positivity were selected. The main underlying conditions were the postoperative state, infection, malignant neoplasm, and autoimmune disease; 18.4% showed an idiopathic onset. FV activity was ≤28％ in all FV inhibitors and major bleeding (MB) was observed in 48.7% of cases. There were no significant differences in MB or mortality among the underlying conditions. The main therapies for bleeding were fresh frozen plasma and platelet concentrate, and the main immunosuppressive therapies were prednisolone, cyclophosphamide and intravenous immunoglobulin. There were no differences in laboratory data between FV inhibitors with and without MB or between survivors and non-survivors. The mortality rate was 14.8% (23.3% in FV inhibitor with MB vs. 10.0% in FV inhibitor without MB; odds ratio 2.732) and the median survival period was 0.8 months. Conclusion: FV activity was ≤28% in all patients with FV inhibitor. While half of the patients did not have MB, the risk of dying was 2-fold higher in those with MB.

Keywords

• Factor V
• Inhibitor
• Acquired immune FV deficiency
• Supplement therapy
• Immunosuppressive therapy