Document Detail

The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation.
MedLine Citation:
PMID:  16704433     Owner:  NLM     Status:  MEDLINE    
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.
Charles R M Hay; S Brown; P W Collins; D M Keeling; R Liesner
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Publication Detail:
Type:  Journal Article; Practice Guideline; Review    
Journal Detail:
Title:  British journal of haematology     Volume:  133     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-05-17     Completed Date:  2006-11-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  England    
Other Details:
Languages:  eng     Pagination:  591-605     Citation Subset:  IM    
University Department of Haematology, Manchester Royal Infirmary, Oxford Road, Manchester, UK.
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MeSH Terms
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
Immune Tolerance
Isoantibodies / blood
Reg. No./Substance:
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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