FEIBA in the treatment of acquired haemophilia A: results from the prospective multicentre French 'FEIBA dans l'hémophilie A acquise' (FEIBHAC) registry.

Abstract

Factor VIII inhibitor bypass activity (FEIBA) is a recommended first-line bypassing agent for bleeding episodes in patients with acquired haemophilia A (AHA). Due to the low incidence of AHA, available clinical data on FEIBA treatment are limited. The study aim was to delineate practice patterns in FEIBA treatment of AHA patients, the haemostatic efficacy of FEIBA, including criteria for its assessment, and safety. A prospective registry was established of AHA patients receiving FEIBA for bleeding episodes or prophylaxis at the time of invasive procedures. Data were collected at 16 participating centres in France. Patients were followed up for 3 months. Haemostatic efficacy, FEIBA regimen and FEIBA-related adverse events were documented. Thirty-four patients averaging 81.8 years old with standard deviation (SD) 8.1 years were included in the study: 33 for acute bleeding and one for haematoma evacuation. The mean initial dose of FEIBA for acute bleeding was 75.4 U kg(-1) (SD, 7.7 U kg(-1) ), most often administered twice daily, and the median duration of FEIBA treatment was 4.0 days (interquartile range, 2.2-8.0 days). FEIBA was effective in managing 88.0% of bleeding episodes (95% confidence interval, 75.8-94.5%). No baseline variables influencing treatment response could be identified. The sensitivity and specificity of an objective haemostatic efficacy scale in predicting sequential investigator assessments of haemostatic efficacy were 45.3% and 84.1% respectively. Four patients experienced a total of six serious adverse events possibly related to FEIBA. In the first prospective study specifically focused on FEIBA treatment of patients with AHA, 88.0% of bleeding episodes were effectively managed.

• Authors: J-Y Borg, C Négrier, I Durieu, E Dolimier, A-M Masquelier, H Lévesque, FEIBHAC Study Group
• Journal: Haemophilia : the official journal of the World Federation of Hemophilia (Haemophilia) Vol. 21 Issue 3 Pg. 330-337 (May 2015) ISSN: 1365-2516 [Electronic] England
• PMID: 25359571 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 John Wiley & Sons Ltd.

Chemical References

• Blood Coagulation Factor Inhibitors
• Blood Coagulation Factors
• Isoantibodies
• Recombinant Proteins
• prothrombin complex concentrates
• Factor VIII
• recombinant FVIIa
• Factor VIIa

Topics

• Aged
• Aged, 80 and over
• Blood Coagulation Factor Inhibitors (blood, immunology)
• Blood Coagulation Factors (administration & dosage, adverse effects, therapeutic use)
• Factor VIII (immunology)
• Factor VIIa (administration & dosage, adverse effects, drug effects)
• Female
• France
• Hemophilia A (complications, diagnosis, drug therapy, immunology)
• Hemorrhage (etiology, therapy)
• Humans
• Isoantibodies (blood, immunology)
• Male
• Prospective Studies
• Recombinant Proteins (administration & dosage, adverse effects, drug effects)
• Registries
• Risk Factors
• Severity of Illness Index
• Treatment Outcome