The use of factor VIII/von Willebrand factor concentrate for immune tolerance induction in haemophilia A patients with high-titre inhibitors: association of clinical outcome with inhibitor epitope profile

Haemophilia ◽  
2008 ◽  
Vol 14 (2) ◽  
pp. 295-302 ◽  
Author(s):  
D. A. GRENINGER ◽  
J. M. SAINT-REMY ◽  
M. JACQUEMIN ◽  
A. BENHIDA ◽  
D. M. DIMICHELE
Keyword(s):  
Clinical Outcome ◽  
Von Willebrand Factor ◽  
Immune Tolerance ◽  
Tolerance Induction ◽  
High Titre ◽  
Haemophilia A ◽  
Immune Tolerance Induction ◽  
Factor Concentrate ◽  
Von Willebrand ◽  
Willebrand Factor

Von Willebrand factor-containing factor VIII concentrates and inhibitors in haemophilia A

2010 ◽  
Vol 104 (11) ◽  
pp. 931-940 ◽  
Author(s):  
Giuseppe Lippi ◽  
Massimo Franchini
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Immune Tolerance ◽  
High Titer ◽  
Tolerance Induction ◽  
Haemophilia A ◽  
Immune Tolerance Induction ◽  
Bleeding Episodes ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryThe development of inhibitors that neutralise the function of factor VIII (FVIII) is currently not only the most challenging complication associated with the treatment of haemophilia A but it also increases the disease-related morbidity as bleeding episodes do not respond to standard therapy. The main short-term goal of the treatment of inhibitor patients is to control bleeding episodes while the long-term one is to permanently eradicate the inhibitor by immune tolerance induction, particularly in the case of high-titer antibodies. Due to some in vitro studies and clinical observations, some investigators have suggested that FVIII concentrates containing von Willebrand factor (VWF) may be less immunogenic than high-purity or recombinant FVIII products. It has also been suggested that success rates for immune tolerance induction are higher when plasma-derived FVIII products are used. The currently available data from laboratory and clinical studies on the role of VWF in inhibitor development and eradication in haemophilia A is critically analysed in this review. As a result, we have not found definitive evidence supporting a role for product type on inhibitor incidence and inhibitor eradication in haemophilia A patients.

Immune tolerance therapy utilizing factor VIII/von Willebrand factor concentrate in haemophilia A patients with high titre factor VIII inhibitors

Haemophilia ◽  
2007 ◽  
Vol 0 (0) ◽  
pp. 071018054549002-???
Author(s):  
M. A. H. KURTH ◽  
D. DIMICHELE ◽  
C. SEXAUER ◽  
J. M. SANDERS ◽  
M. TORRES ◽  
...  
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Immune Tolerance ◽  
High Titre ◽  
Haemophilia A ◽  
Factor Concentrate ◽  
Von Willebrand ◽  
Willebrand Factor
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