| The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. | |
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MedLine Citation:
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PMID: 16704433 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The revised UKHCDO factor (F) VIII/IX Inhibitor Guidelines (2000) are presented. A schema is proposed for inhibitor surveillance, which varies according to the severity of the haemophilia and the treatment type and regimen used. The methodological and pharmacokinetic approach to inhibitor surveillance in congenital haemophilia has been updated. Factor VIII/IX genotyping of patients is recommended to identify those at increased risk. All patients who develop an inhibitor should be considered for immune tolerance induction (ITI). The decision to attempt ITI for FIX inhibitors must be carefully weighed against the relatively high risk of reactions and the nephrotic syndrome and the relatively low response rate observed in this group. The start of ITI should be deferred until the inhibitor has declined below 10 Bethesda Units/ml, where possible. ITI should continue, even in resistant patients, where it is well tolerated and so long as there is a convincing downward trend in the inhibitor titre. The choice of treatment for bleeding in inhibitor patients is dictated by the severity of the bleed, the current inhibitor titre, the previous anamnestic response to FVIII/IX, the previous clinical response and the side-effect profile of the agents available. We have reviewed novel dose-regimens and modes of administration of FEIBA (factor VIII inhibitor bypassing activity) and recombinant activated FVII (rVIIa) and the extent to which these agents may be used for prophylaxis and surgery. Bleeding in acquired haemophilia is usually treated with FEIBA or rVIIa. Immunosuppressive therapy should be initiated at the time of diagnosis with Prednisolone 1 mg/kg/d +/- cyclophosphamide. In the absence of a response to these agents within 6 weeks, second-line therapy with Rituximab, Ciclosporin A, or other multiple-modality regimens may be considered. |
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Authors:
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Charles R M Hay; S Brown; P W Collins; D M Keeling; R Liesner |
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Publication Detail:
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Type: Journal Article; Practice Guideline; Review |
Journal Detail:
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Title: British journal of haematology Volume: 133 ISSN: 0007-1048 ISO Abbreviation: Br. J. Haematol. Publication Date: 2006 Jun |
Date Detail:
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Created Date: 2006-05-17 Completed Date: 2006-11-08 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0372544 Medline TA: Br J Haematol Country: England |
Other Details:
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Languages: eng Pagination: 591-605 Citation Subset: IM |
Affiliation:
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University Department of Haematology, Manchester Royal Infirmary, Oxford Road, Manchester, UK. haemophilia@man.ac.uk |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Evidence-Based Medicine Factor IX / antagonists & inhibitors*, therapeutic use Factor VIII / antagonists & inhibitors*, therapeutic use Hemophilia A / drug therapy, etiology, immunology* Hemophilia B / drug therapy, immunology* Hemorrhage / drug therapy Hemostasis, Surgical / methods Humans Immune Tolerance Isoantibodies / blood Male |
| Chemical | |
Reg. No./Substance:
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0/Isoantibodies; 9001-27-8/Factor VIII; 9001-28-9/Factor IX |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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