Literature DB >> 17498074

A systematic approach to controlling problem bleeds in patients with severe congenital haemophilia A and high-titre inhibitors.

J Teitel1, E Berntorp, P Collins, R D'Oiron, B Ewenstein, E Gomperts, J Goudemand, A Gringeri, N Key, C Leissinger, P Monahan, G Young.   

Abstract

UNLABELLED: The presence of inhibitory antibodies to clotting factors complicates the treatment of bleeding in haemophilia patients. For patients with high-titre inhibitors, bypassing agents are essential to haemostatic management. To determine optimal treatment practices, an international panel of physicians convened to develop a systematic treatment approach for problem bleeds (i.e. bleeds that are unresponsive to initial therapy with a single agent within a reasonable amount of time) in haemophilia patients with inhibitors. AIM: The goal of this panel was to develop a consensus algorithm that would aid physicians in considering a variety of treatment approaches to optimize patient care by preventing extensive therapy with inadequate treatments that may lead to suboptimal patient outcomes and unnecessary costs.
METHODS: Consensus opinions were analyzed for clinical preferences at different time periods, depending on patient response to treatment. Decision-making points were defined based on the type of bleed: every 8-12 h for the first 24 h, then every 24 h thereafter for limb-threatening bleeds; every 2-4 h for 2-7 days for life-threatening bleeds.
RESULTS: The resultant consensus guidelines provide a generalized methodology to guide the treatment of problem bleeds in patients with severe haemophilia A and inhibitors, and emphasize changing treatment at the first sign of an inadequate haemostatic response. The treatment algorithms apply to both paediatric and adult patients, although the differences between the two groups were reviewed.
CONCLUSION: These guidelines are focused on optimising the timing of treatment decisions, which may lead to faster responses and improved outcomes.

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Year:  2007        PMID: 17498074     DOI: 10.1111/j.1365-2516.2007.01449.x

Source DB:  PubMed          Journal:  Haemophilia        ISSN: 1351-8216            Impact factor:   4.287


  9 in total

1.  Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management.

Authors:  Massimo Franchini; Giancarlo Castaman; Antonio Coppola; Cristina Santoro; Ezio Zanon; Giovanni Di Minno; Massimo Morfini; Elena Santagostino; Angiola Rocino
Journal:  Blood Transfus       Date:  2015-06-24       Impact factor: 3.443

2.  Emicizumab use in major orthopedic surgery.

Authors:  Craig D Seaman; Margaret V Ragni
Journal:  Blood Adv       Date:  2019-06-11

3.  Modeling costs and outcomes associated with a treatment algorithm for problem bleeding episodes in patients with severe hemophilia a and high-titer inhibitors.

Authors:  Patrick Bonnet; Alessandro Gringeri; Edward Gomperts; Cindy Anne Leissinger; Roseline d'Oiron; Jerome Teitel; Guy Young; Meg Franklin; Bruce Ewenstein; Erik Berntorp
Journal:  Am Health Drug Benefits       Date:  2011-07

4.  Sequential bypassing agents during major orthopedic surgery: a new approach to hemostasis.

Authors:  Craig D Seaman; Margaret V Ragni
Journal:  Blood Adv       Date:  2017-07-18

5.  Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding.

Authors:  Antonio Coppola; Massimo Franchini; Armando Tripodi; Rita C Santoro; Giancarlo Castaman; Renato Marino; Ezio Zanon; Cristina Santoro; Gianna F Rivolta; Laura Contino; Raimondo De Cristofaro; Angelo C Molinari; Paolo Gresele; Angiola Rocino
Journal:  Blood Transfus       Date:  2022-01-20       Impact factor: 5.752

6.  Transplanted endothelial cells repopulate the liver endothelium and correct the phenotype of hemophilia A mice.

Authors:  Antonia Follenzi; Daniel Benten; Phyllis Novikoff; Louisa Faulkner; Sanj Raut; Sanjeev Gupta
Journal:  J Clin Invest       Date:  2008-03       Impact factor: 14.808

Review 7.  Treatment and Prevention of Bleeds in Haemophilia Patients with Inhibitors to Factor VIII/IX.

Authors:  Angiola Rocino; Massimo Franchini; Antonio Coppola
Journal:  J Clin Med       Date:  2017-04-17       Impact factor: 4.241

8.  The efficacy of bypassing agents in surgery of hemophilia patients with inhibitors.

Authors:  Hee Young Ju; Hye Lim Jang; Young Shil Park
Journal:  Blood Res       Date:  2015-09-22

9.  Sequential therapy with activated prothrombin complex concentrates and recombinant activated factor VII to treat unresponsive bleeding in patients with hemophilia and inhibitors: a single center experience.

Authors:  Myung Hee Han; Young Shil Park
Journal:  Blood Res       Date:  2013-12-24
  9 in total

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