Document Detail


Successful immune tolerance induction with high-dose coagulation factor VIII and intravenous immunoglobulins in a patient with congenital hemophilia and high-titer inhibitor of coagulation factor VIII despite unfavorable prognosis for the therapy.
MedLine Citation:
PMID:  19488019     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The production of factor VIII (FVIII) inhibitors is a serious problem of replacement therapy with FVIII concentrates in hemophiliacs. It affects 10-20% patients and leads to an increased risk of severe bleeding and its complications. Immune tolerance induction (ITI) is considered the appropriate treatment in such cases, despite different regimens without clearly defined effectiveness. ITI eradicates FVIII inhibitors and allows retreatment with FVIII concentrates in 70% of patients. CASE REPORT: The case of a patient with congenital hemophilia A in whom allo-antibodies against FVIII were identified in a high titer at the age of 5 after 70 exposures to human plasma FVIII concentrates is presented. A spontaneous decrease in inhibitor titer to 14 BU/ml within 6 months after the termination of FVIII administration allowed ITI, consisting of FVIII in high doses and intravenous immunoglobulins. Cessation of bleeding during the treatment was achieved with recombinant activated FVII (rFVIIa). ITI lasted for 22 months and, despite the high inhibitor titer at the start of ITI suggesting poor outcome, it led to eradication of the inhibitor. The prophylactic replacement therapy with FVIII was restarted and since then no signs of FVIII inhibitor have been observed. CONCLUSIONS: ITI with high-dose FVIII, intravenous immunoglobulins, and rFVIIa is a beneficial treatment option for hemophiliac A patients with high-titer FVIII inhibitor.
Authors:
Peter Kubisz; Ivana Plamenová; Pavol Hollý; Ján Stasko
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Medical science monitor : international medical journal of experimental and clinical research     Volume:  15     ISSN:  1643-3750     ISO Abbreviation:  Med. Sci. Monit.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-02     Completed Date:  2009-07-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9609063     Medline TA:  Med Sci Monit     Country:  Poland    
Other Details:
Languages:  eng     Pagination:  CS105-11     Citation Subset:  IM    
Affiliation:
National Hemostasis and Thrombosis Center, Clinic of Hematology and Transfusion Medicine, Jessenius Faculty of Medicine of the Comenius University, Faculty Hospital, Martin, Slovakia. kubisz@jfmed.uniba.sk
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MeSH Terms
Descriptor/Qualifier:
Blood Coagulation Factor Inhibitors / administration & dosage,  pharmacology,  therapeutic use
Child
Dose-Response Relationship, Drug
Drug Administration Schedule
Factor VIII / administration & dosage*,  antagonists & inhibitors,  therapeutic use*
Hemophilia A / diagnosis*,  immunology,  therapy*
Hemostasis
Humans
Immune Tolerance / immunology*
Immunoglobulins, Intravenous / administration & dosage*,  therapeutic use*
Immunotherapy
Prognosis
Time Factors
Chemical
Reg. No./Substance:
0/Blood Coagulation Factor Inhibitors; 0/Immunoglobulins, Intravenous; 9001-27-8/Factor VIII

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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