Clinical experience of factor XI deficiency: the role of fresh frozen plasma and factor XI concentrate

Abstract

Factor XI deficiency is a rare autosomally transmitted coagulopathy that is associated with a variable bleeding tendency. Recently there have been reports of thrombotic events following the administration of a virally inactivated factor XI concentrate (BPL) to factor XI deficient patients. We have therefore reviewed a single centre's experience of the use of factor XI concentrate over a 6-year period and compared this to our previous experience of either no treatment or treatment with fresh frozen plasma (FFP) in 103 patients. There were 156 procedures performed without haemo- static cover. The incidence of bleeding was greatest following tonsillectomy (71%) and dental extraction (Sl'h). There was a trend for bleeding complications to be associated with lower levels of factor XI but patients with all levels of factor XI suffered bleeding complica- tions. There were 38 procedures carried out under FFP cover, with only one patient suffering excessive bleeding and no serious complications. Factor XI concentrate was given to 25 patients to cover 45 episodes. There were no bleeding complications. Three patients suffered serious complications. One patient, with a previous history of cardiovascular disease, died of a myocardial infarction and a second had an ischaemic episode resulting in a %day hospital admission. These episodes both occurred on the same day as the factor XI infusion. A third patient suffered bilateral pulmonary emboli 7 weeks after a prolonged course of factor XI concentrate. These finding suggest that factor XI concentrate should be contraindicated in patients with a history of cardiovascular disease, when FFP should be used. Guide- lines for the use of factor XI concentrates should be revised, and work performed to establish the mechanism of these thrombotic events.

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

“…However, large volumes of FFP may require to be administered to achieve therapeutic levels, which can lead to fluid overload. FFP given as a dose of 15-20 mL kg )1 appears effective [154,155]. Monitoring of the response is important and a virally inactivated product is recommended.…”

Sample conditions determine the ability of thrombin generation parameters to identify bleeding phenotype in FXI deficiency

“…High rates of bleeding have been reported in FXI deficiency in association with these procedures. 2,4,19 Bleeders (n 5 24) were defined as those requiring blood product transfusion or return to theater/ dentist for resuturing or packing. Nonbleeders (n 5 50) were those who had not experienced excessive bleeding.…”

Factor XI deficiency—resolving the enigma?

“…Subsequent studies have confirmed this finding. [4][5][6] This disorder is, therefore, not completely recessive and the variability of the bleeding tendency constitutes one enigma of this deficiency.…”