{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T15:34:13Z","timestamp":1792251253263,"version":"4.4.0"},"reference-count":33,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2013,4,8]],"date-time":"2013-04-08T00:00:00Z","timestamp":1365379200000},"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":[[2013,12]]},"abstract":"Background<\/jats:title>Thrombotic thrombocytopenic purpura (TTP<\/jats:styled-content>) is characterized by deficient ADAMTS<\/jats:styled-content>13 activity. Treatment involves plasma exchange (PE<\/jats:styled-content>). Both fresh\u2010frozen plasma (FFP<\/jats:styled-content>) and cryosupernatant (CSP<\/jats:styled-content>) are used, but it remains to be determined which is more effective.<\/jats:p><\/jats:sec>Study Design and Methods<\/jats:title>To analyze the interaction between von Willebrand factor (VWF<\/jats:styled-content>) and ADAMTS<\/jats:styled-content>13, we used large\u2010pore isoelectric focusing (IEF<\/jats:styled-content>) analysis followed by detection with anti\u2010ADAMT<\/jats:styled-content>S13 monoclonal antibody. FFP<\/jats:styled-content>, CSP<\/jats:styled-content>, cryoprecipitate (CP<\/jats:styled-content>), and purified ADAMTS<\/jats:styled-content>13 were analyzed for their effects on high shear stress\u2013induced platelet aggregation (H<\/jats:styled-content>\u2010SIPA<\/jats:styled-content>).<\/jats:p><\/jats:sec>Results<\/jats:title>IEF<\/jats:styled-content> analysis of normal plasma revealed three groups of ADAMTS<\/jats:styled-content>13 bands with pI<\/jats:styled-content> of 4.9 to 5.6, 5.8 to 6.7, and 7.0 or 7.5. Two band groups (pI<\/jats:styled-content> 4.9\u20105.6 and 5.8\u20106.7) were found in plasma of a patient with T<\/jats:styled-content>ype 3 von W<\/jats:styled-content>illebrand disease, in which VWF<\/jats:styled-content> is absent, whereas no bands were found in plasma of a patient with congenital ADAMTS<\/jats:styled-content>13 deficiency. Mixing these plasmas generated the bands at pI<\/jats:styled-content> 7.0 or 7.5, representing the VWF<\/jats:styled-content>\u2010ADAMTS<\/jats:styled-content>13 complex; these bands were absent in CSP<\/jats:styled-content>. FFP<\/jats:styled-content> and purified ADAMTS<\/jats:styled-content>13 down regulated H<\/jats:styled-content>\u2010SIPA<\/jats:styled-content> in a dose\u2010dependent manner. However, CP<\/jats:styled-content> did not inhibit H<\/jats:styled-content>\u2010SIPA<\/jats:styled-content> in the initial phase, and the degree of inhibition at the endpoint was almost indistinguishable from those of the other two plasma products.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>Both plasma products (FFP<\/jats:styled-content> and CSP<\/jats:styled-content>) are effective for PE<\/jats:styled-content> in TTP<\/jats:styled-content> patients. However, CSP<\/jats:styled-content> may be more favorable, because it has lower levels of VWF<\/jats:styled-content> and almost normal ADAMTS<\/jats:styled-content>13 activity, but lower levels of ADAMTS<\/jats:styled-content>13 in complex with larger VWF<\/jats:styled-content> multimers.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.12182","type":"journal-article","created":{"date-parts":[[2013,4,8]],"date-time":"2013-04-08T09:14:46Z","timestamp":1365412486000},"page":"3192-3202","source":"Crossref","is-referenced-by-count":20,"title":["ADAMTS<\/scp>13 unbound to larger von W<\/scp>illebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment"],"prefix":"10.1111","volume":"53","author":[{"given":"Yuji","family":"Hori","sequence":"first","affiliation":[{"name":"Department of Blood Transfusion Medicine Nara Medical University Kashihara Nara Japan"},{"name":"The Chemo\u2010Sero\u2010Therapeutic Research 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