Cost-effectiveness of recombinant activated factor VII vs. plasma-derived activated prothrombin complex concentrate in the treatment of mild-to-moderate bleeding episodes in patients with severe haemophilia A and inhibitors in Spain

Haemophilia ◽  
2013 ◽  
Vol 19 (6) ◽  
pp. 841-846 ◽  
Author(s):  
V. Jimenez-Yuste ◽  
R. Núñez ◽  
J. A. Romero ◽  
B. Montoro ◽  
B. Espinós
Reumatismo ◽  
2019 ◽  
Vol 71 (1) ◽  
pp. 37-41 ◽  
Author(s):  
E. Mauro ◽  
E. Garlatti Costa ◽  
A. Zanier ◽  
M. Maset ◽  
A. Ermacora ◽  
...  

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Here we report the different therapeutic approaches in 4 cases of elderly patients with AHA and co-morbidities.


Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 369-374 ◽  
Author(s):  
Peter W. Collins

Abstract Management of acquired hemophilia A is challenging and should be undertaken in close collaboration with a hemophilia center with expertise in the field. Treatment involves controlling and preventing bleeds and using immunosuppression to eradicate the inhibitor. Prompt diagnosis is important to allow early hemostatic treatment and to prevent nonessential invasive procedures. First-line hemostatic treatment should be with a bypassing agent. Recombinant activated factor VII and the activated prothrombin complex concentrate anti-inhibitor coagulant complex (Factor Eight Inhibitor Bypassing Activity, or FEIBA) but equally efficacious but both associated with thrombotic events when used in acquired hemophilia. Immunosuppression should be started as soon as a diagnosis has been confirmed. The combination of steroids and cyclophosphamide may induce more patients into remission than steroids alone. Current data do not suggest that rituximab results in better outcomes. Relapse is common (10%-20%) in the first 6 months after immunosuppression is stopped, and patients need to be followed up regularly to allow early diagnosis and treatment of relapse.


2011 ◽  
Vol 07 (02) ◽  
pp. 140 ◽  
Author(s):  
Emanuela Marchesini ◽  
Domenico Prisco ◽  
Alfonso Iorio ◽  
◽  
◽  
...  

The development of inhibitors remains the most challenging complication of treatment in persons with haemophilia, resulting in increased morbidity and a significant economic burden. The ultimate goal of treatment in patients with inhibitors is immune tolerance induction (ITI) therapy; however, during the induction phase of ITI, when ITI fails and where ITI is not affordable, the treatment of bleeding becomes a crucial issue. Recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC) have been developed to bypass the inhibitor antibody effect and have been tested in several randomised controlled trials, including two crossover head-to-head comparisons. Two systematic reviews of the literature have appraised and synthesised the available evidence. The recombinant drug seems to provide a more favourable benefit–risk ratio and may be easily administered as a single front-loaded bolus, making it a good candidate for the role of first-line treatment for bleeding in patients with inhibitors. Aggressive treatment of acute bleeds should be considered, including the use of higher and repeated-dose regimens until complete resolution of the bleed.


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