ADAMTS13 antibody and inhibitor assays

Moore, G.
Vetr, H.
Binder, N.
Moore, G.
Vetr, H.
Binder, N.

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Publication date

January 2023

DOI

10.1007/978-1-0716-3175-1_36

Publisher

Humana

Abstract

A finding of an ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level of <10% of normal is usually sufficient to distinguish thrombotic thrombocytopenic purpura (TTP) from other thrombotic microangiopathies. TTP can be congenital or acquired, the most common form being acquired immune-mediated TTP caused by autoantibodies than inhibit ADAMTS13 function and/or increase its clearance. Basic 1 + 1 mixing tests can detect the presence of inhibitory antibodies, and quantification can be achieved with Bethesda-type assays that measure loss of function in a series of mixtures of test plasma and normal plasma. Not all patients present with inhibitory antibodies, and here the ADAMTS13 deficiency...