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Scheiflinger, F. et al.

Scheiflinger, Knöbl, Trattner, Plaimauer, Mohr, Dockal, Dorner, Rieger,

Nonneutralizing IgM and IgG antibodies to von Willebrand factor- cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura.

Acquired thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by autoantibodies inhibitory to ADAMTS-13. We report data on a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely used fluid phase activity assay. With a newly developed enzyme-linked immunosorbent assay, using immobilized recombinant ADAMTS-13, we found high titers of IgM and IgG antibodies that bound to ADAMTS-13, but did not neutralize protease activity. These autoantibodies probably influenced the half-life of ADAMTS-13 or its binding to the endothelial cell surface, thereby compromising ADAMTS-13 activity in vivo. Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution, and the epitope mapping of nonneutralizing antibodies will be an important area for future research.[1]

References

Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Scheiflinger, F., Knöbl, P., Trattner, B., Plaimauer, B., Mohr, G., Dockal, M., Dorner, F., Rieger, M. Blood (2003) [Pubmed]

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