Document Detail

Immunosuppressive agents in the treatment of inhibitors in congenital haemophilia A and B - a systematic literature review.
MedLine Citation:
PMID:  24957105     Owner:  NLM     Status:  In-Data-Review    
The development of inhibitory antibodies to factor VIII (FVIII) or factor IX (FIX) in patients with haemophilia is a serious complication of treatment with coagulation factor concentrates. Antibodies develop in 10-15% of haemophilia A and in up to 5% of haemophilia B patients. Several strategies have been developed over the years to facilitate the eradication of inhibitors and reduce the cost. These include plasmapheresis and/or extracorporeal protein A absorption to remove the inhibitor from the plasma, and immunosuppression and/or immune modulation to suppress the production of inhibitory antibodies. Different immunosuppressive (IS) agents have been described with varying success. To evaluate the outcome of these agents, we performed a systematic literature review using the PubMed database. The total number of articles identified was 345; 299 papers were excluded leaving 46 papers to be included in the study. No randomised studies were identified, only case reports and case series. The most frequently used agents in the 46 case reports and cohort studies identified were cyclophosphamide and rituximab. All cases exposed to cyclophosphamide, rituximab and other IS agents had a complete success rate of 40-44%, 40-63% and 33-56%, respectively. However, the definition of success was not consistent among the studies. In conclusion, our review of the literature indicates that IS agents in combination with FVIII or FIX could be an option and may be cost-effective in many patients. The risk of adverse events seems to be relatively low. To fully explore the effect of IS agents, randomised studies are warranted.
Britta Antonia Petra Laros-van Gorkom; Céline Falaise; Jan Astermark
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  European journal of haematology. Supplementum     Volume:  76     ISSN:  0902-4506     ISO Abbreviation:  Eur J Haematol Suppl     Publication Date:  2014 Aug 
Date Detail:
Created Date:  2014-06-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8703474     Medline TA:  Eur J Haematol Suppl     Country:  England    
Other Details:
Languages:  eng     Pagination:  26-38     Citation Subset:  IM    
Copyright Information:
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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