{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T04:28:30Z","timestamp":1790051310988,"version":"4.0.1"},"reference-count":32,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2007,6,26]],"date-time":"2007-06-26T00:00:00Z","timestamp":1182816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2007,9]]},"abstract":"<jats:p><jats:bold>BACKGROUND:<\/jats:bold> Little is known about the pathophysiology of human immunodeficiency virus (HIV)\u2010related thrombotic thrombocytopenic purpura (TTP). It is generally assumed that acquired ADAMTS13 deficiency is due to the presence of autoantibody inhibitors, but limited data are available regarding ADAMTS13 activity and inhibitors in such patients.<\/jats:p><jats:p><jats:bold>STUDY DESIGN AND METHODS:<\/jats:bold> By use of a collagen\u2010binding assay, ADAMTS13 activity was analyzed at presentation in 20 patients with HIV\u2010related TTP. The presence of inhibitors in patients with reduced ADAMTS13 activity was assessed with mixing studies. The correlation between ADAMTS13 activity and inhibitors and other laboratory and clinical parameters was assessed.<\/jats:p><jats:p><jats:bold>RESULTS:<\/jats:bold> The patients fell clearly into two groups with regard to ADAMTS13 activity. Six patients (30%) had activity within the normal range, whereas the remaining 14 patients had severely reduced levels. Of the patients with reduced activity, only 5 patients had a detectable inhibitor whereas 8 showed no evidence of an inhibitor. There was significant correlation between normal ADAMTS13 activity and lower CD4 counts (p\u2003=\u20030.049). von Willebrand factor (VWF) antigen levels were significantly higher in patients with reduced ADAMTS13 activity (p\u2003=\u20030.03). Low activity in the absence of a detectable inhibitor was associated with significantly higher D\u2010dimer levels (p\u2003=\u20030.01) and worse clinical outcome.<\/jats:p><jats:p><jats:bold>CONCLUSION:<\/jats:bold> The heterogeneity with regard to ADAMTS13 activity and the absence of inhibitors in the majority of patients indicate that other factors are important in the pathogenesis of HIV\u2010related TTP. VWF release and localized coagulation activation due to direct viral or cytokine\u2010mediated endothelial cell injury is likely to be playing a major role.<\/jats:p>","DOI":"10.1111\/j.1537-2995.2007.01346.x","type":"journal-article","created":{"date-parts":[[2007,6,26]],"date-time":"2007-06-26T23:35:20Z","timestamp":1182900920000},"page":"1710-1716","source":"Crossref","is-referenced-by-count":60,"title":["ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus\u2013related thrombotic thrombocytopenic 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