sábado, 28 de abril de 2018

European principles of inhibitor management in patients with haemophilia | Orphanet Journal of Rare Diseases | Full Text

European principles of inhibitor management in patients with haemophilia | Orphanet Journal of Rare Diseases | Full Text



European principles of inhibitor management in patients with haemophilia

  • P. L. F. GiangrandeEmail authorView ORCID ID profile,
  • C. Hermans,
  • B. O’Mahony,
  • P. de Kleijn,
  • M. Bedford,
  • A. Batorova,
  • J. Blatný,
  • K. Jansone and
  • on behalf of the European Haemophilia Consortium (EHC) and the European Association for Haemophilia and Allied Disorders (EAHAD)
Orphanet Journal of Rare Diseases201813:66
Received: 5 December 2017
Accepted: 6 April 2018
Published: 27 April 2018

Abstract

Background

In spite of recent major advances in the understanding and treatment of inhibitor development in patients with haemophilia, multidisciplinary management of many of these patients remains suboptimal and highly heterogenous across Europe.

Methods

Following a series of multidisciplinary meetings and a review of the literature, the European haemophilia community of health professionals and patients jointly defined practical optimum standards for ensuring and harmonizing treatment and care for patients with an inhibitor.

Results

Ten complementary principles for the management of inhibitors in haemophilia have been developed, emphasizing the importance and benefits of a centralized, multidisciplinary, expert and holistic approach.

Conclusions

This document will serve as a benchmark to improve the multidisciplinary and practical management of patients with inhibitor. Implementation and adherence to each of these principles should have a major positive impact on the management and outcomes of patients developing an inhibitor.

Keywords

HaemophiliaGuidelinesInhibitorsFactor VIIIFactor IXBypassing agentsImmune tolerance

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