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Factor VIII-hydrolyzing IgG in acquired and congenital hemophilia |
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| Authors: | Bharath Wootla Ankit Mahendra Jordan D Dimitrov Alain Friboulet Desirazu N Rao Jeanne-Yvonne Borg Srinivas V Kaveri Sébastien Lacroix-Desmazes |
| Institution: | a Centre de Recherche des Cordeliers, Université Pierre et Marie Curie - Paris6, UMR S 872, Paris F-75006, France b Université Paris Descartes, UMR S 872, Paris F-75006, France c INSERM, U872, Paris F-75006, France d Université de Technologie de Compiègne, CNRS UMR 6022 Génie Enzymatique et Cellulaire, BP 20529 60205 Compiègne Cedex, France e Université de Caen, Laboratoire d’Hématologie EA 3212, Caen F-14000, France f Department of Biochemistry, Indian Institute of Science, Bangalore 560 012, India g Applied Biochemistry, Faculty of Engineering, Oita University, Oita 870-1192, Japan h Rouen University Hospital, Rouen F-76031, France |
| Abstract: | Anti-factor VIII (FVIII) inhibitory IgG may arise as alloantibodies to therapeutic FVIII in patients with congenital hemophilia A, or as autoantibodies to endogenous FVIII in individuals with acquired hemophilia. We have described FVIII-hydrolyzing IgG both in hemophilia A patients with anti-FVIII IgG and in acquired hemophilia patients. Here, we compared the properties of proteolytic auto- and allo-antibodies. Rates of FVIII hydrolysis differed significantly between the two groups of antibodies. Proline-phenylalanine-arginine-methylcoumarinamide was a surrogate substrate for FVIII-hydrolyzing autoantibodies. Our data suggest that populations of proteolytic anti-FVIII IgG in acquired hemophilia patients are different from that of inhibitor-positive hemophilia A patients. |
| Keywords: | Catalytic antibody Acquired hemophilia Hemophilia A FVIII inhibitor Factor VIII PFR-MCA |
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