Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study

Zanon, Ezio; Milan, Marta; Gamba, G; Ambaglio, Chiara; Saggiorato, Graziella; Spiezia, Luca; Montani, Nadia; Prandoni, Paolo

doi:10.1016/j.thromres.2015.10.032

Cited by 23 publications

(25 citation statements)

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“…The short half-life of recombinant factor VIIa is limiting the use of this agent in prophylaxis. Activated prothrombin complex concentrate has been used for secondary prophylaxis with an apparent reduction in bleeding [ 16 , 17 ]. However, this approach may require several infusions per week, probably not feasible in many AHA patients over prolonged periods of time.…”

Section: Discussionmentioning

confidence: 99%

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

2020

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Immunosuppressive therapy (IST) is administered to patients with acquired hemophilia A (AHA) to eradicate autoantibodies against coagulation factor VIII (FVIII). Data from registries previously demonstrated that IST is often complicated by adverse events, in particular infections. This pilot study was set out to assess the feasibility of reduced-intensity, risk factor–stratified IST. We followed a single-center consecutive cohort of twenty-five patients with AHA receiving IST according to a new institutional treatment standard. Based on results from a previous study, GTH-AH 01/2020, patients were stratified into “poor risk” (FVIII < 1 IU/dl or inhibitor ≥ 20 Bethesda units (BU)/ml) or “good risk” (FVIII ≥ 1 IU/dl and inhibitor < 20 BU/ml). Outcomes were compared between the current cohort and the GTH registry as a historic control (n = 102). Baseline characteristics of the cohort were not different from the historic control. Partial remission, defined as FVIII recovered to > 50 IU/dl, was achieved by 68% of patients after a median time of 112 days, which was lower and significantly later than in the historic control (hazard ratio: 1.8, 95% confidence interval 1.2–2.8). Complete remission, overall survival, and frequency of fatal infections were not different. Grade 3 and 4 infections were more frequent. The impact of risk factors that was observed in the historic cohort was no longer apparent, as partial and complete remission and overall survival were similar in “good risk” and “poor risk” patients. In conclusion, reduced-intensity, risk factor–stratified IST is feasible in AHA but did not decrease the risk of infections and mortality in this cohort.

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Section: Discussionmentioning

confidence: 99%

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

2020

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“…43 APCC has also been used for secondary prophylaxis. 44,45 In a retrospective study, APCC exhibited a favorable safety profile indicating that it is well-tolerated with few adverse events. 46 Thrombotic events, including myocardial infarction and venous thrombosis, were mostly reported in patients with additional risk factors.…”

Section: Activated Prothrombin Complex Concentratementioning

confidence: 99%

International recommendations on the diagnosis and treatment of acquired hemophilia A

et al. 2020

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A cquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged activated partial thromboplastin time should never be ignored prior to invasive procedures. Control of acute bleeding and prevention of injuries that may provoke bleeding are top priorities in patients with AHA. We recommend treatment with bypassing agents, including recombinant activated factor VII, activated prothrombin complex concentrate, or recombinant porcine FVIII in bleeding patients. Autoantibody eradication can be achieved with immunosuppressive therapy, including corticosteroids, cyclophosphamide and rituximab, or combinations thereof. The median time to remission is 5 weeks, with considerable interindividual variation. FVIII activity at presentation, inhibitor titer and autoantibody isotype are prognostic markers for remission and survival. Comparative clinical studies to support treatment recommendations for AHA do not exist; therefore, we provide practical consensus guidance based on recent registry findings and the authors' clinical experience in treating patients with AHA.

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“…The study collected data from January 2003 to December 2012 for the retrospective group, and from January 2013 to December 2015 for the prospective one. Fifty‐six patients were included in the registry, seven of whom had been included in a previous study (Zanon et al , ).…”

Section: Baseline Characteristics and The Treatment Of Patients Enrolmentioning

confidence: 99%

Activated prothrombin complex concentrate (FEIBA^®) in acquired haemophilia A: a large multicentre Italian study – the FAIR Registry

et al. 2018

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Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study

Cited by 23 publications

References 20 publications

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

International recommendations on the diagnosis and treatment of acquired hemophilia A

Activated prothrombin complex concentrate (FEIBA^®) in acquired haemophilia A: a large multicentre Italian study – the FAIR Registry

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Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study

Cited by 23 publications

References 20 publications

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

International recommendations on the diagnosis and treatment of acquired hemophilia A

Activated prothrombin complex concentrate (FEIBA®) in acquired haemophilia A: a large multicentre Italian study – the FAIR Registry

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Product

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Activated prothrombin complex concentrate (FEIBA^®) in acquired haemophilia A: a large multicentre Italian study – the FAIR Registry