Low-dose immune tolerance induction for paediatric haemophilia patients with factor VIII inhibitors

Simple item page
dc.contributor.authorUnuvar, Ayşegül
dc.contributor.authorKavaklı, Kaan
dc.contributor.authorKazancı, Elif Güler
dc.contributor.authorSaylı, Tülin Revide
dc.contributor.authorÖren, Hale
dc.contributor.authorCelkan, Tulin Tiraje
dc.contributor.authorGürsel, Türkiz
dc.contributor.buuauthorBaytan, Birol
dc.contributor.departmentUludağ Üniversitesi/Tıp Fakültesi/Pediatrik Hematoloji Anabilim Dalı.tr_TR
dc.contributor.scopusid6506622162tr_TR
dc.date.accessioned2022-04-21T10:37:40Z
dc.date.available2022-04-21T10:37:40Z
dc.date.issued2008-03
dc.description.abstractThe development of an inhibitor against factor VIII (FVIII) is a serious complication in children with haemophilia A. Immune tolerance induction (ITI) therapy is generally considered to be the best approach to eradicate the inhibitor. In this paper, the low-dose (<= 50 IU kg(-1) twice or three times weekly with plasma-derived factor concentrates) ITI regimen used in Turkey is discussed. This regimen was given to 21 haemophilia A patients with high titer inhibitors. The median age at the beginning of ITI was 9 years and exposure days were 25. The median pre-ITI historical peak inhibitor titer, and inhibitor titer when ITI started were 80 BU (range 6.0-517), 19.2 BU (range 3.6-515), respectively. Complete immune tolerance was defined as the time at which at least two negative inhibitor assays was obtained with no anamnestic response. Our two cases were not reached in follow-up period. Immune tolerance could be achieved in 5 of 19 (26.3%) patients within a median time of 6 months. Partial tolerance was obtained in 7 patients while treatment failed in spite of significant decreased inhibitor levels in the other patients. A relapse developed in one immune-tolerized patient, one year later. The level of inhibitor titer at the beginning of ITI (<= 10 BU), the pre-ITI historical peak inhibitor titer (< 50 BU), and the time between the first diagnosis inhibitor to starting ITI (< 12 months) were main factors in the success (complete or partial tolerance) of ITI. In conclusion, the outcome of low-dose ITI protocol was not satisfactory in this retrospective study.en_US
dc.identifier.citationÜnüvar, A. vd. (2008). "Low-dose immune tolerance induction for paediatric haemophilia patients with factor VIII inhibitors". Haemophilia, 14(2), 315-322.en_US
dc.identifier.endpage322tr_TR
dc.identifier.issn1351-8216
dc.identifier.issn1365-2516
dc.identifier.issue2tr_TR
dc.identifier.pubmed18081830tr_TR
dc.identifier.scopus2-s2.0-40349096477tr_TR
dc.identifier.startpage315tr_TR
dc.identifier.urihttps://doi.org/10.1111/j.1365-2516.2007.01621.x
dc.identifier.urihttps://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2007.01621.x
dc.identifier.urihttp://hdl.handle.net/11452/25954
dc.identifier.volume14tr_TR
dc.identifier.wos000253626100012tr_TR
dc.indexed.pubmedPubMeden_US
dc.indexed.scopusScopusen_US
dc.indexed.wosSCIEen_US
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.collaborationYurt içitr_TR
dc.relation.collaborationSanayitr_TR
dc.relation.journalHaemophiliaen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergitr_TR
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectHematologyen_US
dc.subjectChildrenen_US
dc.subjectHaemophiliaen_US
dc.subjectImmune toleranceen_US
dc.subjectInhibitoren_US
dc.subjectLow-doseen_US
dc.subjectFactor-ixen_US
dc.subjectA patientsen_US
dc.subjectRegistryen_US
dc.subjectAntibodiesen_US
dc.subjectDiagnosisen_US
dc.subject.emtreeActivated prothrombin complexen_US
dc.subject.emtreeBeriate pen_US
dc.subject.emtreeBlood clotting factor 8en_US
dc.subject.emtreeBlood clotting factor 8 antibodyen_US
dc.subject.emtreeBlood clotting factor 8 concentrateen_US
dc.subject.emtreeBblood clotting factor 8 inhibitoren_US
dc.subject.emtreeCyclophosphamideen_US
dc.subject.emtreeFactor 8 yen_US
dc.subject.emtreeHaemofil men_US
dc.subject.emtreeKoate dvien_US
dc.subject.emtreeOctanateen_US
dc.subject.emtreeProthrombin complexen_US
dc.subject.emtreeRecombinant blood clotting factor 7aen_US
dc.subject.emtreeUnclassified drugen_US
dc.subject.emtreeAdjuvant therapyen_US
dc.subject.emtreeAdolescenten_US
dc.subject.emtreeArticleen_US
dc.subject.emtreeBleedingen_US
dc.subject.emtreeChilden_US
dc.subject.emtreeChild healthen_US
dc.subject.emtreeClinical articleen_US
dc.subject.emtreeControlled studyen_US
dc.subject.emtreeDrug substitutionen_US
dc.subject.emtreeDrug withdrawalen_US
dc.subject.emtreeHemophiliaen_US
dc.subject.emtreeHumanen_US
dc.subject.emtreeImmunological toleranceen_US
dc.subject.emtreeLow drug doseen_US
dc.subject.emtreeOutcome assessmenten_US
dc.subject.emtreePriority journalen_US
dc.subject.emtreeRelapseen_US
dc.subject.emtreeRetrospective studyen_US
dc.subject.emtreeTreatment durationen_US
dc.subject.emtreeTreatment failureen_US
dc.subject.emtreeTurkey (republic)en_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshAutoantibodiesen_US
dc.subject.meshChi-square distributionen_US
dc.subject.meshChilden_US
dc.subject.meshChild, preschoolen_US
dc.subject.meshDrug administration scheduleen_US
dc.subject.meshFactor VIIIen_US
dc.subject.meshHemophilia aen_US
dc.subject.meshHumansen_US
dc.subject.meshImmune toleranceen_US
dc.subject.meshInfanten_US
dc.subject.meshRecurrenceen_US
dc.subject.meshRetrospective studiesen_US
dc.subject.meshRisk factorsen_US
dc.subject.meshTreatment outcomeen_US
dc.subject.meshTurkeyen_US
dc.subject.scopusHemophilia A; Recombinant Blood Clotting Factor 8; Patient with Hemophiliaen_US
dc.subject.wosHematologyen_US
dc.titleLow-dose immune tolerance induction for paediatric haemophilia patients with factor VIII inhibitorsen_US
dc.typeArticle
dc.wos.quartileQ3en_US

Files

License bundle
Now showing 1 - 1 of 1
Name:
license.txt
Size:
1.71 KB
Format:
Item-specific license agreed upon to submission
Description:
Download

Collections

Web of Science
PubMed
Scopus