Document Detail

The long-term course of factor VIII inhibitors in patients with congenital haemophilia A without immune tolerance induction.
MedLine Citation:
PMID:  21057702     Owner:  NLM     Status:  MEDLINE    
The development of alloantibodies that inhibit or neutralise the function of factor VIII is considered the most serious complication of the treatment of congenital haemophilia A. In order to describe their course without immune tolerance induction (ITI), we documented data on all performed inhibitor tests with dates as well as on clotting factor infusions of all consecutive patients who were treated in our centre between 1993 and 2006. Patients were tested every 7.1 months (95% confidence interval [CI], 6.6-7.8). A 'sustained negative inhibitor status' was defined as consistent non-positive inhibitor measurements for two years or longer. A total of 60/486 (12%) patients tested had a positive inhibitor titre in two or more occasions. Most of the patients (56%) with a maximum inhibitor titre of < 5 Bethesda unit (BU)/ml (named "low titre inhibitor") developed a sustained negative inhibitor status. Among patients with high (5-9.9 BU/ml) and very high (≥ 10 BU/ml) inhibitor titres, the proportions were 50% and 3%, respectively. Our findings suggest that ITI might not be needed for all patients with non-transient inhibitors, especially when their maximum inhibitor titre is below 10 BU/ml. Further studies in countries where ITI is not available are needed to examine predictors of the natural sustained negative inhibitor status.
Camila Caram; Roberta Grazielle de Souza; Júlio Carepa de Sousa; Tatiana Araújo Pereira; Ana Maria do Amaral Cerqueira; Johanna G van der Bom; Suely Meireles Rezende
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-11-05
Journal Detail:
Title:  Thrombosis and haemostasis     Volume:  105     ISSN:  0340-6245     ISO Abbreviation:  Thromb. Haemost.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-01-04     Completed Date:  2011-05-18     Revised Date:  2011-05-23    
Medline Journal Info:
Nlm Unique ID:  7608063     Medline TA:  Thromb Haemost     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  59-65     Citation Subset:  IM    
Grupo de Estudo a Hemostasia e Trombose -- GETHe/UFMG, Belo Horizonte, Brazil.
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MeSH Terms
Blood Coagulation Factors / therapeutic use
Child, Preschool
Factor VIII / immunology*
Hemophilia A / immunology*,  therapy
Immune Tolerance
Isoantibodies / blood*
Longitudinal Studies
Retrospective Studies
Young Adult
Reg. No./Substance:
0/Blood Coagulation Factors; 0/Isoantibodies; 9001-27-8/Factor VIII

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

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