| ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor. | |
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MedLine Citation:
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PMID: 19404519 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Inhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive agents like mycophenolat mofetil (MMF) or rituximab have been reported. We present a case of successful inhibitor elimination with a combined immune-modulating therapy and high-dose factor IX (FIX). This boy had developed a FIX inhibitor at the age of 5 years and had a history of allergic reactions to FIX and to FEIBAO. Under on-demand treatment with recombinant activated FVII the inhibitor became undetectable but the boy suffered from multiple joint and muscle bleeds. At the age of 11.5 years ITI was attempted with a combination of rituximab, MMF, dexamethasone, intravenous immunoglobulins and high-dose FIX. The inhibitor did not reappear and FIX half-life normalized. No allergic reaction, no signs of nephrotic syndrome and no serious infections were observed. |
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Authors:
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Karin Beutel; H Hauch; J Rischewski; U Kordes; J Schneppenheim; Reinhard Schneppenheim |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Hämostaseologie Volume: 29 ISSN: 0720-9355 ISO Abbreviation: Hamostaseologie Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-04-30 Completed Date: 2009-07-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8204531 Medline TA: Hamostaseologie Country: Germany |
Other Details:
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Languages: eng Pagination: 155-7 Citation Subset: IM |
Affiliation:
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Paediatric Haematology and Oncology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany. beutel@uke.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Antibodies, Monoclonal
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therapeutic use Child Dexamethasone / therapeutic use Factor IX / therapeutic use* Hemophilia B / immunology* Humans Immune Tolerance* Immunosuppressive Agents / therapeutic use* Infant Male Mycophenolic Acid / analogs & derivatives*, therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Antibodies, Monoclonal; 0/Immunosuppressive Agents; 0/rituximab; 128794-94-5/mycophenolate mofetil; 24280-93-1/Mycophenolic Acid; 50-02-2/Dexamethasone; 9001-28-9/Factor IX |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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