Abstract
in four samples of patients with hereditary TTP (no. 2, 15, 19and 30) no inhibitor was found by FRETS-VWF73. In theremaining six samples tested for ADAMTS-13 inhibitors, adefinite inhibitor was found in one sample (no. 23), while fivesamples (no. 1, 12, 22, 25 and 27) tested negative. These sixsamples had been discordantly classified as positive, uncertain,or negative for inhibitor by the other assays [10].We conclude that the FRETS-VWF73 assay is an easy-to-perform, reliable ADAMTS-13 activity assay. The advantageovertheotherADAMTS-13activityassaysistheavailabilityofresults within 1–2 h. From data shown by Dong et al. [12], theinter-individual variation of ADAMTS-13 activity in normalsubjects seems to be most pronounced the shorter a patient’splasmaisincubatedwiththeVWFsubstrate.Therefore,itisnotexcluded that ADAMTS-13 activity determined by the initialrateassay,FRETS-VWF73,maydifferfromthatdeterminedbyotherassaysusingaprolongedincubation(16–20 h)ofpatient’sADAMTS-13 with the VWF substrate. Nevertheless, in theseries of samples analyzed here, ADAMTS-13 activity deter-mined by FRETS-VWF73 assay was in good accordance withthatmeasuredbytheotherassays.Thedefinitevalueofthisnewassay remains to be determined in a large cohort of patientsdiagnosed with acquired or hereditary TTP.AcknowledgementsThis work was supported by grants from the Swiss NationalFoundation for Scientific Research (3200B0-108261) and theMach-Gaensslen Foundation, Switzerland.References
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