Abstract
: In acquired haemophilia A (AHA), risk for recurrent bleeding exists until the inhibitor is detectable. Thus, patients with persisting inhibitor may benefit from prophylaxis with activated prothrombin complex concentrate (aPCC). Potential thromboembolic complications and cost are also factors to consider. Today, no high level evidence or clear recommendations are available on aPCC prophylaxis in AHA. Recently, a small prospective study demonstrated a favourable outcome with short-term, daily administered aPCC infusion. Here we report a retrospective case series of 19 patients with AHA to demonstrate our practice on aPCC prophylaxis. In our practice, clinical bleeding tendency guided our decision on the initiation of aPCC prophylaxis. In patients with serious bleeding tendency, aPCC infusion was prolonged beyond bleeding resolution in a twice-weekly or thrice-weekly regimen. Serious bleeding phenotype included a single episode of life-threatening bleeding or recurrent, severe haemorrhages. Patients who did not present such events were treated on-demand. The preventive dose of aPCC was equal with the lowest effective therapeutic dose. Prophylaxis was continued until the inhibitor disappeared. Eleven patients received aPCC prophy...Continue Reading
References
Nov 25, 2003·Journal of Thrombosis and Haemostasis : JTH·K VáradiP L Turecek
Oct 19, 2006·Blood·Peter W CollinsUNKNOWN UK Haemophilia Centre Doctors' Organisation
Jul 10, 2008·Thrombosis and Haemostasis·Péter IlonczaiZoltán Boda
Apr 2, 2009·Haematologica·Angela Huth-KühneJean St-Louis
Sep 29, 2009·Haemophilia : the Official Journal of the World Federation of Hemophilia·D PerryT T Yee
Nov 4, 2011·The New England Journal of Medicine·Cindy LeissingerLorenzo Mantovani
Feb 11, 2012·Journal of Thrombosis and Haemostasis : JTH·P KnoeblUNKNOWN EACH2 Registry Contributors
Apr 21, 2012·Blood·Peter CollinsUNKNOWN EACH2 registry collaborators
May 24, 2012·Blood·Francesco BaudoUNKNOWN EACH2 registry contributors
Aug 14, 2012·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Ánita ArokszállásiAgota Schlammadinger
Apr 12, 2013·Haemophilia : the Official Journal of the World Federation of Hemophilia·J Y BorgUNKNOWN SACHA Study Group
Jul 31, 2013·British Journal of Haematology·Peter W CollinsUNKNOWN United Kingdom Haemophilia Centre Doctors' Organization
Aug 6, 2013·Haemophilia : the Official Journal of the World Federation of Hemophilia·S V AntunesW-Y Wong
Nov 2, 2014·Haemophilia : the Official Journal of the World Federation of Hemophilia·J-Y BorgUNKNOWN FEIBHAC Study Group
Jan 28, 2015·Haemophilia : the Official Journal of the World Federation of Hemophilia·R Kruse-JarresB Ewenstein
Oct 28, 2015·Thrombosis Research·Ezio ZanonPaolo Prandoni
Feb 2, 2016·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Claude NegrierUNKNOWN FEIBA PASS Study group
Citations
Sep 14, 2019·Hematology·Naifang YeWenman Wu
Jul 6, 2020·Annals of Hematology·Christiane DobbelsteinAndreas Tiede
Dec 12, 2020·Journal of Thrombosis and Haemostasis : JTH·Andreas TiedePaul Knöbl