Titlebook: Inhibitors to Coagulation Factors; Louis M. Aledort,Leon W. Hoyer,Gilbert C. White Book 1995 The Editor(s) (if applicable) and The Author(

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Antibodies to von Willebrand factor in von Willebrand diseases first reported in 2 consecutive studies by Sarji et al. (1974) and Stratton et al. (1975). After this, 14 additional cases of alloantibodies were described and reviewed by Mannucci and Mari (1984). A survey carried out on behalf of the World Federation of Hemophilia identified 6 additional cases t

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T-cell receptors in immune responsesions, most recognize antigenic peptide presented by molecules encoded within the major histocompatibility complex (MHC). The fine specificity of a T-cell is determined by a heterodimeric receptor for antigen (TCR) displayed on the cell surface. The TCR present on the majority of T-cells is composed

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Immunoglobulin heavy chain variable region gene usage in human autoimmune diseasesomosome, are separated by thousands of base pairs. The repertoire of germline variable region genes represents the substrate upon which diversity is generated in the antibody system, since the recombination of one among different available V., D, and J., as well as V. and J. gene segments, provides

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Porcine factor VIII therapy in patients with factor VIII inhibitorsis drawn partly from the published literature and partly from an international survey of the use of porcine F.VIII in patients with congenital hemophilia conducted under the auspices of the ISTH F.VIII and IX scientific standardization subcommittee. The treatment of congenital and acquired hemophili

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Clinical update on the use of recombinant factor VIIly of factor VIII (F.VIII) or factor IX (RIX). Such an agent should be not only hemostatically active but also safe with respect to thromboembolic complications. Factor Vila (F.VIIa) becomes proteolytically active when complexed with tissue factor or other phopholipids minimizing the risk of inducin

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Immunogenetics of the human immune response to factor VIIIors both at the F.VIII locus and at other loci, possibly associated with the immune response, play a role in inhibitor production. This brief review will try to summarize some of the more recent information on how 1) the structure of the F.VIII protein, 2) the nature of F.VIII mutation that results