Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study.
Despite anti-haemorrhagic therapy with proper doses of activated prothrombin complex concentrate (aPCC, Feiba®), patients with acquired haemophilia A (AHA) have a considerable risk of recurrent bleeding complications. Evidence in support of the benefit-to-risk ratio of prevention strategies with the use of lower doses of aPCC following the end of the initial treatment period is scarce and inconclusive. We report our experience in the management of 18 consecutive patients with AHA admitted to two Haemophilia centres in Italy. We managed the first 11 according to current guidelines (e.g., with conventional aPCC doses until bleeding resolution). Then, we decided to prolong the treatment beyond bleeding resolution with lower doses of the same concentrate (short-term prophylaxis) in the 7 additional patients. In these patients, the treatment was continued for as long as the titre of FVIII inhibitor was found to decrease by at least 50% when compared to the baseline one. We observed six relapses of bleeding in patients in whom aPCC was confined to the treatment of the qualifying bleeding episode, and none in patients to whom lower doses were administered until the pre-specified decrease in the titre of FVIII inhibitor was achieved. No patients experienced thrombotic complications during the study period. Prolonging the treatment with lower doses of aPCC beyond the initial phase in patients with AHA in whom the titre of FVIII inhibitor is still high is likely to safely prevent further bleeding complications.
Department of Cardiac Thoracic and Vascular Sciences, Haemophilia Centre Unit of Coagulopathies, University Hospital of Padua, Italy. Electronic address: ezio.zanon@unipd.it.
Ezio
E
Zanon
Department of Cardiac Thoracic and Vascular Sciences, Haemophilia Centre Unit of Coagulopathies, University Hospital of Padua, Italy.
Marta
M
Milan
Department of Internal Medicine and Medical Therapy, Diseases of Haemostasis, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Gabriella
G
Gamba
Department of Internal Medicine and Medical Therapy, Diseases of Haemostasis, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Chiara
C
Ambaglio
Department of Internal Medicine, University Hospital of Padua, Italy.
Graziella
G
Saggiorato
Department of Internal Medicine, University Hospital of Padua, Italy.
Luca
L
Spiezia
Department of Internal Medicine and Medical Therapy, Diseases of Haemostasis, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Nadia
N
Montani
Department of Cardiac Thoracic and Vascular Sciences, Haemophilia Centre Unit of Coagulopathies, University Hospital of Padua, Italy.
Paolo
P
Prandoni
D000368:Aged; D000369:Aged, 80 and over; D019774:Blood Coagulation Factor Inhibitors / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D003029:Coagulants / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy* / Q000276:immunology; D006470:Hemorrhage / Q000276:immunology / Q000517:prevention & control*; D006801:Humans; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012042:Registries; D013923:Thromboembolism / Q000097:blood / Q000276:immunology
D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D003029:Coagulants; C025667:prothrombin complex concentrates; D005169:Factor VIII; C065655:anti-inhibitor coagulant complex
Acquired haemophilia; Activated prothrombin complex concentrate; Bleeding; Prophylaxis