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Non-Genetic Risk Factors and the Development of Inhibitors in Haemophilia: a Comprehensive Review and Consensus Report

2010-09-01Journal article Open access
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dc.contributor.authorAstermark, J
dc.contributor.authorAltisent, C
dc.contributor.authorBatorova, A
dc.contributor.authorDiniz, MJ
dc.contributor.authorGringeri, A
dc.contributor.authorHolme, PA
dc.contributor.authorKarafoulidou, A
dc.contributor.authorLopez-Fernández, MF
dc.contributor.authorReipert, BM
dc.contributor.authorRocino, A
dc.contributor.authorSchiavoni, M
dc.contributor.authorvon Depka, M
dc.contributor.authorWindyga, J
dc.contributor.authorFijnvandraat, K
dc.date.accessioned2017-04-19T09:44:54Z
dc.date.available2017-04-19T09:44:54Z
dc.date.issued2010-09-01
dc.description.abstractThe development of inhibitors to the infused factor in patients with haemophilia is a serious clinical problem. Recent evidence suggests that alongside the strong genetic contribution to inhibitor formation, there are a number of non-genetic factors--perceived by the immune system as danger signals--which promote formation of inhibitors. This study provides a comprehensive review of clinical studies relating to these factors and also presents a survey of opinion concerning their importance and clinical influence, conducted among the members of the European Haemophilia Treatment Standardisation Board (EHTSB). Taken together, this information highlights the lack of robust data concerning the influence of several non-genetic risk factors on inhibitor development, and an urgent need for prospective, well-conducted studies that adhere to recommendations made by the European Medicines Agency (EMEA) for studying inhibitors. Based on current literature, the EHTSB formulated consensus recommendations. It is desirable to minimize intensive treatment wherever possible, given the clinical situation. Prophylaxis should be offered to all children, although we still need to determine optimal dosing with respect to inhibitor development, and age for starting treatment. Vaccinations should be given subcutaneously and concomitant factor concentrate infusions avoided. According to the board, there is no evidence in the literature supporting suggestions that the type of concentrate influences inhibitor risk; but all patients should be monitored during their first exposures. Furthermore, there is no evidence to support an association between pregnancy-related issues, breast feeding and treatment-related factors (e.g. route of administration, or use of blood components) and inhibitor development.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationHaemophilia. 2010 Sep 1;16(5):747-66pt_PT
dc.identifier.doi10.1111/j.1365-2516.2010.02231.xpt_PT
dc.identifier.urihttp://hdl.handle.net/10400.17/2673
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBlackwell Publishingpt_PT
dc.subjectAge Factorspt_PT
dc.subjectBreast Feedingpt_PT
dc.subjectDelivery, Obstetricpt_PT
dc.subjectFactor VIIIpt_PT
dc.subjectFemalept_PT
dc.subjectHemophilia Apt_PT
dc.subjectHumanspt_PT
dc.subjectMalept_PT
dc.subjectPregnancypt_PT
dc.subjectRisk Factorspt_PT
dc.subjectBlood Coagulation Factor Inhibitorspt_PT
dc.subjectHSJ IMUpt_PT
dc.titleNon-Genetic Risk Factors and the Development of Inhibitors in Haemophilia: a Comprehensive Review and Consensus Reportpt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage766pt_PT
oaire.citation.issue5pt_PT
oaire.citation.startPage747pt_PT
oaire.citation.titleHaemophiliapt_PT
oaire.citation.volume16pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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