Loading…
The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation
Summary 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 su...
Saved in:
Published in: | British journal of haematology 2006-06, Vol.133 (6), p.591-605 |
---|---|
Main Authors: | , , , , |
Format: | Article |
Language: | English |
Subjects: | |
Citations: | Items that this one cites Items that cite this one |
Online Access: | Get full text |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
cited_by | cdi_FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3 |
---|---|
cites | cdi_FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3 |
container_end_page | 605 |
container_issue | 6 |
container_start_page | 591 |
container_title | British journal of haematology |
container_volume | 133 |
creator | Hay, Charles R. M. Brown, S. Collins, P. W. Keeling, D. M. Liesner, R. |
description | Summary
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. |
doi_str_mv | 10.1111/j.1365-2141.2006.06087.x |
format | article |
fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_67978857</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1062336721</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3</originalsourceid><addsrcrecordid>eNqNkcFu1DAQhi0EotvCKyALCW4b7DiJEyQOsAU2UKmXFnGzxo6961Vib-1EtK_AU-PsrqjECV9szXzza6wPIUxJRtN5t8soq8plTgua5YRUGalIzbP7J2jxt_EULQghfElJUZ-h8xh3hFBGSvocndGKk6JgbIF-32w17ixsnI82YnAdHsDBRg_ajdgbbECNPuAfbdseuu1PbN3WSpuq8T0GvJlsp3vrNDbBD3hMebfOjrrD363bdKm0Bj34_db2FvAqxQaNL_2cGvF12ICzEUbr3Qv0zEAf9cvTfYFuv3y-Wa2XV9df29XHq6UqeJH-Y0oKZUlMaVRJi4ZRDqqDqiFKVUC4MdBIqcvCGEVyJmXOJTBJCc0lk51kF-jtMXcf_N2k4ygGG5Xue3DaT1FUvOF1XfIEvv4H3PkpuLSboE1dUVbQPEH1EVLBxxi0EftgBwgPghIxyxI7MTsRsxMxyxIHWeI-jb465U9y0N3j4MlOAt6cAIgKehPAKRsfOc55Qw-Lfjhyv2yvH_57AfHp23p-sT-lRLCw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>198613412</pqid></control><display><type>article</type><title>The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation</title><source>Wiley-Blackwell Read & Publish Collection</source><creator>Hay, Charles R. M. ; Brown, S. ; Collins, P. W. ; Keeling, D. M. ; Liesner, R.</creator><creatorcontrib>Hay, Charles R. M. ; Brown, S. ; Collins, P. W. ; Keeling, D. M. ; Liesner, R.</creatorcontrib><description>Summary
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.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/j.1365-2141.2006.06087.x</identifier><identifier>PMID: 16704433</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Biological and medical sciences ; diagnosis ; Evidence-Based Medicine ; Factor IX - antagonists & inhibitors ; Factor IX - therapeutic use ; Factor VIII - antagonists & inhibitors ; Factor VIII - therapeutic use ; factor VIII/IX inhibitors ; Hematologic and hematopoietic diseases ; Hematology ; Hemophilia A - drug therapy ; Hemophilia A - etiology ; Hemophilia A - immunology ; Hemophilia B - drug therapy ; Hemophilia B - immunology ; Hemorrhage - drug therapy ; Hemostasis, Surgical - methods ; Humans ; Immune Tolerance ; Isoantibodies - blood ; Male ; management ; Medical sciences ; Platelet diseases and coagulopathies</subject><ispartof>British journal of haematology, 2006-06, Vol.133 (6), p.591-605</ispartof><rights>2006 INIST-CNRS</rights><rights>Copyright Blackwell Publishing Jun 2006</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3</citedby><cites>FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=17779157$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16704433$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hay, Charles R. M.</creatorcontrib><creatorcontrib>Brown, S.</creatorcontrib><creatorcontrib>Collins, P. W.</creatorcontrib><creatorcontrib>Keeling, D. M.</creatorcontrib><creatorcontrib>Liesner, R.</creatorcontrib><title>The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary
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.</description><subject>Biological and medical sciences</subject><subject>diagnosis</subject><subject>Evidence-Based Medicine</subject><subject>Factor IX - antagonists & inhibitors</subject><subject>Factor IX - therapeutic use</subject><subject>Factor VIII - antagonists & inhibitors</subject><subject>Factor VIII - therapeutic use</subject><subject>factor VIII/IX inhibitors</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hematology</subject><subject>Hemophilia A - drug therapy</subject><subject>Hemophilia A - etiology</subject><subject>Hemophilia A - immunology</subject><subject>Hemophilia B - drug therapy</subject><subject>Hemophilia B - immunology</subject><subject>Hemorrhage - drug therapy</subject><subject>Hemostasis, Surgical - methods</subject><subject>Humans</subject><subject>Immune Tolerance</subject><subject>Isoantibodies - blood</subject><subject>Male</subject><subject>management</subject><subject>Medical sciences</subject><subject>Platelet diseases and coagulopathies</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><recordid>eNqNkcFu1DAQhi0EotvCKyALCW4b7DiJEyQOsAU2UKmXFnGzxo6961Vib-1EtK_AU-PsrqjECV9szXzza6wPIUxJRtN5t8soq8plTgua5YRUGalIzbP7J2jxt_EULQghfElJUZ-h8xh3hFBGSvocndGKk6JgbIF-32w17ixsnI82YnAdHsDBRg_ajdgbbECNPuAfbdseuu1PbN3WSpuq8T0GvJlsp3vrNDbBD3hMebfOjrrD363bdKm0Bj34_db2FvAqxQaNL_2cGvF12ICzEUbr3Qv0zEAf9cvTfYFuv3y-Wa2XV9df29XHq6UqeJH-Y0oKZUlMaVRJi4ZRDqqDqiFKVUC4MdBIqcvCGEVyJmXOJTBJCc0lk51kF-jtMXcf_N2k4ygGG5Xue3DaT1FUvOF1XfIEvv4H3PkpuLSboE1dUVbQPEH1EVLBxxi0EftgBwgPghIxyxI7MTsRsxMxyxIHWeI-jb465U9y0N3j4MlOAt6cAIgKehPAKRsfOc55Qw-Lfjhyv2yvH_57AfHp23p-sT-lRLCw</recordid><startdate>200606</startdate><enddate>200606</enddate><creator>Hay, Charles R. M.</creator><creator>Brown, S.</creator><creator>Collins, P. W.</creator><creator>Keeling, D. M.</creator><creator>Liesner, R.</creator><general>Blackwell Publishing Ltd</general><general>Blackwell</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>200606</creationdate><title>The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation</title><author>Hay, Charles R. M. ; Brown, S. ; Collins, P. W. ; Keeling, D. M. ; Liesner, R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2006</creationdate><topic>Biological and medical sciences</topic><topic>diagnosis</topic><topic>Evidence-Based Medicine</topic><topic>Factor IX - antagonists & inhibitors</topic><topic>Factor IX - therapeutic use</topic><topic>Factor VIII - antagonists & inhibitors</topic><topic>Factor VIII - therapeutic use</topic><topic>factor VIII/IX inhibitors</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hematology</topic><topic>Hemophilia A - drug therapy</topic><topic>Hemophilia A - etiology</topic><topic>Hemophilia A - immunology</topic><topic>Hemophilia B - drug therapy</topic><topic>Hemophilia B - immunology</topic><topic>Hemorrhage - drug therapy</topic><topic>Hemostasis, Surgical - methods</topic><topic>Humans</topic><topic>Immune Tolerance</topic><topic>Isoantibodies - blood</topic><topic>Male</topic><topic>management</topic><topic>Medical sciences</topic><topic>Platelet diseases and coagulopathies</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hay, Charles R. M.</creatorcontrib><creatorcontrib>Brown, S.</creatorcontrib><creatorcontrib>Collins, P. W.</creatorcontrib><creatorcontrib>Keeling, D. M.</creatorcontrib><creatorcontrib>Liesner, R.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hay, Charles R. M.</au><au>Brown, S.</au><au>Collins, P. W.</au><au>Keeling, D. M.</au><au>Liesner, R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2006-06</date><risdate>2006</risdate><volume>133</volume><issue>6</issue><spage>591</spage><epage>605</epage><pages>591-605</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><coden>BJHEAL</coden><abstract>Summary
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.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>16704433</pmid><doi>10.1111/j.1365-2141.2006.06087.x</doi><tpages>15</tpages><oa>free_for_read</oa></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0007-1048 |
ispartof | British journal of haematology, 2006-06, Vol.133 (6), p.591-605 |
issn | 0007-1048 1365-2141 |
language | eng |
recordid | cdi_proquest_miscellaneous_67978857 |
source | Wiley-Blackwell Read & Publish Collection |
subjects | Biological and medical sciences diagnosis Evidence-Based Medicine Factor IX - antagonists & inhibitors Factor IX - therapeutic use Factor VIII - antagonists & inhibitors Factor VIII - therapeutic use factor VIII/IX inhibitors Hematologic and hematopoietic diseases Hematology Hemophilia A - drug therapy Hemophilia A - etiology Hemophilia A - immunology Hemophilia B - drug therapy Hemophilia B - immunology Hemorrhage - drug therapy Hemostasis, Surgical - methods Humans Immune Tolerance Isoantibodies - blood Male management Medical sciences Platelet diseases and coagulopathies |
title | The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation |
url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-23T00%3A59%3A21IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=The%20diagnosis%20and%20management%20of%20factor%20VIII%20and%20IX%20inhibitors:%20a%20guideline%20from%20the%20United%20Kingdom%20Haemophilia%20Centre%20Doctors%20Organisation&rft.jtitle=British%20journal%20of%20haematology&rft.au=Hay,%20Charles%20R.%20M.&rft.date=2006-06&rft.volume=133&rft.issue=6&rft.spage=591&rft.epage=605&rft.pages=591-605&rft.issn=0007-1048&rft.eissn=1365-2141&rft.coden=BJHEAL&rft_id=info:doi/10.1111/j.1365-2141.2006.06087.x&rft_dat=%3Cproquest_cross%3E1062336721%3C/proquest_cross%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c4747-1f51a550f5fc5149317acda690cc6a07ffa9bbe54ffc023bb27ba3b1012b3bdb3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=198613412&rft_id=info:pmid/16704433&rfr_iscdi=true |