Document Detail

Inhibitors in hemophilia patients: current status and management.
MedLine Citation:
PMID:  7942786     Owner:  NLM     Status:  MEDLINE    
The development of inhibitor antibodies is a long-standing, well-documented complication of coagulation factor replacement therapy and is difficult to treat. Previous estimates of 5-15% of patients developing inhibitors may be low, since newer data suggest a wider range of occurrence. Factors that appear to affect inhibitor development include the severity of hemophilia, age, genetics, and, possibly, the type of replacement therapy administered. Recent studies raise the concern that recombinant factor therapies may be associated with more rapid development and higher levels of inhibitors in previously untreated patients. However, results of different studies are often difficult to compare owing to differences in methodology and populations studied. Further studies standardized in design and methods are clearly needed. Management of patients with inhibitors involves control of acute bleeding episodes and, over the long term, induction of immune tolerance for the coagulation replacement therapy. Many with low or moderate levels of inhibitors may be treated simply by administering higher doses of clotting factor. Other therapies appropriate for those with high levels of inhibitors include porcine F VIII and factor VIII "bypassing" agents, such as recombinant factor VIIa. Long-term immune tolerance has been achieved through the high-dose "Bonn" regimen and immunosuppressive regimens such as the "Malmö" method. Although management of inhibitor patients has improved, it still represents a major challenge. Further research is needed to identify which patients will develop inhibitors and tolerance, as well as to develop better methods to manage, reduce, or eliminate inhibitors from these patients.
L Aledort
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  American journal of hematology     Volume:  47     ISSN:  0361-8609     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  1994 Nov 
Date Detail:
Created Date:  1994-11-18     Completed Date:  1994-11-18     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  208-17     Citation Subset:  IM    
Mount Sinai Medical Center, New York, NY 10029.
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MeSH Terms
Factor IX / antagonists & inhibitors,  immunology
Factor VIII / antagonists & inhibitors,  immunology
Hemophilia A / epidemiology,  immunology*,  therapy*
Immunosuppressive Agents / pharmacology
Isoantibodies / analysis*,  immunology
Reg. No./Substance:
0/Immunosuppressive Agents; 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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