title: Management of bleeding in severe factor V deficiency with a factor V inhibitor.
abstract: Factor V (FV) inhibitor arises rarely after using fresh frozen plasma (FFP) to treat inherited FV deficiency and is often a real therapeutic challenge. Here, we report a patient with a severe FV deficiency who developed such an inhibitor and was then treated with recombinant activated FVII (rFVIIa) and platelet concentrates (PC). Monitoring was assessed by thrombin generation assay (TGA). PC were more effective than rFVIIa in treating bleeding, but there was no correlation between the TGA results and clinical efficacy.
authors: Haemophilia Center, University Hospital, Nantes, France.
authors: L
authors: L
authors: Ardillon
authors: A
authors: A
authors: Lefrançois
authors: J
authors: J
authors: Graveleau
authors: M
authors: M
authors: Fouassier
authors: C
authors: C
authors: Ternisien
authors: M
authors: M
authors: Sigaud
authors: M
authors: M
authors: Fretigny
authors: I
authors: I
authors: Archambeaud
authors: M
authors: M
authors: Trossaërt
mesh_terms: D005165:Factor V / Q000037:antagonists & inhibitors*; D005166:Factor V Deficiency / Q000150:complications* / Q000235:genetics; D015942:Factor VIIa / Q000494:pharmacology*; D006454:Hemoglobins / Q000378:metabolism; D006470:Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense / Q000235:genetics; D010949:Plasma; D011994:Recombinant Proteins / Q000494:pharmacology; D013917:Thrombin / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D006454:Hemoglobins; D011994:Recombinant Proteins; D005165:Factor V; C103587:recombinant FVIIa; D015942:Factor VIIa; D013917:Thrombin
keywords: factor V inhibitor; fresh frozen plasma; platelet concentrate; recombinant activated factor VII; thrombin generation assay