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Thromb Haemost 2016; 115(05): 872-895
DOI: 10.1160/TH15-07-0568
Consensus Paper
Schattauer GmbH

Spanish Consensus Guidelines on prophylaxis with bypassing agents in patients with haemophilia and inhibitors

Maria Fernanda López-Fernández
1   Complejo Hospitalario Universitario A Coruña, Haematology, A Coruña, Spain
,
Carmen Altisent Roca
2   Hospital Universitario Vall d’Hebron, Haemophilia Unit, Barcelona, Spain
,
Maria Teresa Álvarez-Román
3   Hospital Universitario La Paz, Thrombosis and Haemostasis, Madrid, Spain
,
Mariana Isabel Canaro Hirnyk
4   Hospital Universitario Son Espases, Haematology, Palma de Mallorca, Spain
,
Maria Eva Mingot-Castellano
5   Hospital Regional Universitario de Málaga, Haematology, Málaga, Spain
,
Víctor Jiménez-Yuste
3   Hospital Universitario La Paz, Thrombosis and Haemostasis, Madrid, Spain
,
Ana Rosa Cid Haro
6   Hospital Universitario La Fe, Thrombosis and Haemostasis, Valencia, Spain
,
Rosario Pérez-Garrido
7   Hospital Universitario Virgen del Rocío, Haemophilia Unit, Sevilla, Spain
,
Carmen Sedano Balbas
8   Hospital Universitario Marqués de Valdecilla, Haematology, Cantabria, Spain
› Author Affiliations Financial Support: Medical writing support was provided by Maite Artés and Marta Perez from Adelphi and was supported by Baxalta with an unrestricted educational grant. Baxalta does not necessarily endorse, support, or agree with any or all of the content.
Further Information

Publication History

Received: 16 July 2015

Accepted after major revision: 08 January 2016

Publication Date:
06 December 2017 (online)

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Summary

Prophylaxis with the blood clotting factor, factor VIII (FVIII) is ineffective for individuals with haemophilia A and high-titre inhibitors to FVIII. Prophylaxis with the FVIII bypassing agents activated prothrombin complex concentrates (aPCC; FEIBA® Baxalta) or recombinant activated factor VII (rFVIIa; Novo-Seven®, Novo Nordisk) may be an effective alternative. It was our aim to develop evidence -and expert opinion- based guidelines for prophylactic therapy for patients with high-titre inhibitors to FVIII. A panel of nine Spanish haematologists undertook a systematic review of the literature to develop consensusbased guidance. Particular consideration was given to prophylaxis in patients prior to undergoing immune tolerance induction (ITI) (a process of continued exposure to FVIII that can restore sensitivity for some patients), during the ITI period and for those not undergoing ITI or for whom ITI had failed. These guidelines offer guidance for clinicians in deciding which patients might benefit from prophylaxis with FVIII bypassing agents, the most appropriate agents in various clinical settings related to ITI, doses and dosing regimens and how best to monitor the efficacy of prophylaxis. The paper includes recommendations on when to interrupt or stop prophylaxis and special safety concerns during prophylaxis. These consensus guidelines offer the most comprehensive evaluation of the clinical evidence base to date and should be of considerable benefit to clinicians facing the challenge of managing patients with severe haemophilia A with high-titre FVIII inhibitors.

Keywords

Prophylaxis - bypassing agents - haemophilia - inhibitors - guidelines
 

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