Factor VIII-hydrolyzing IgG in acquired and congenital hemophilia.
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.
Wootla
Bharath
B
Centre de Recherche des Cordeliers, Université Pierre et Marie Curie, UMR S 872, Paris, France.
Mahendra
Ankit
A
Dimitrov
Jordan D
JD
Friboulet
Alain
A
Borel-Derlon
Annie
A
Rao
Desirazu N
DN
Uda
Taizo
T
Borg
Jeanne-Yvonne
JY
Bayry
Jagadeesh
J
Kaveri
Srinivas V
SV
Lacroix-Desmazes
Sébastien
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017151:Antibodies, Catalytic / Q000097:blood / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D005169:Factor VIII / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000235:genetics / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007518:Isoantibodies / Q000097:blood / Q000276:immunology; D008297:Male; D008875:Middle Aged
D017151:Antibodies, Catalytic; D001323:Autoantibodies; D007074:Immunoglobulin G; D007518:Isoantibodies; D005169:Factor VIII