{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T15:05:33Z","timestamp":1792508733162,"version":"4.5.0"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2013,12,28]],"date-time":"2013-12-28T00:00:00Z","timestamp":1388188800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"funder":[{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Complement dysregulation is key in the pathogenesis of atypical <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>aemolytic <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>raemic <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>yndrome (a<jats:styled-content style=\"fixed-case\">HUS<\/jats:styled-content>), but no clear role for complement has been identified in <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>hrombotic <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>hrombocytopenic <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>urpura (<jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>). We aimed to assess complement activation and cytokine response in acute antibody\u2010mediated <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>. Complement <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>3a and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>5a and cytokines (interleukin (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>)\u20102, <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20104, <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20106, <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201010, tumour necrosis factor, interferon\u2010\u03b3 and IL\u201017a) were measured in 20 acute <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> patients and 49 remission cases. Anti\u2010<jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 immunoglobulin <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>) subtypes were measured in acute patients in order to study the association with complement activation. In acute <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>, median <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>3a and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>5a were significantly elevated compared to remission, <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>3a 63\u00b79\u00a0ng\/ml vs. 38\u00b72\u00a0ng\/ml (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001) and C5a 16\u00b74\u00a0ng\/ml vs. 9\u00b729\u00a0ng\/ml (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001), respectively. Median <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20106 and <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201010 levels were significantly higher in the acute vs. remission groups, <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20106: 8\u00a0pg\/ml vs. 2\u00a0pg\/ml (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7003), IL\u201010: 6\u00a0pg\/ml vs. 2\u00a0pg\/ml (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001). C3a levels correlated with both anti\u2010<jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> (<jats:italic>r<\/jats:italic><jats:sub>s<\/jats:sub>\u00a0=\u00a00\u00b7604, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7017) and IL\u201010 (<jats:italic>r<\/jats:italic><jats:sub>s<\/jats:sub>\u00a0=\u00a00\u00b7692, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7006). No anti\u2010<jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> subtype was associated with higher complement activation, but patients with the highest <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>3a levels had 3 or 4 <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> subtypes present. These results suggest complement anaphylatoxin levels are higher in acute <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> cases than in remission, and the complement response seen acutely may relate to anti\u2010<jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> antibody and <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201010 levels.<\/jats:p>","DOI":"10.1111\/bjh.12707","type":"journal-article","created":{"date-parts":[[2013,12,28]],"date-time":"2013-12-28T08:24:43Z","timestamp":1388219083000},"page":"858-866","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["Complement and cytokine response in acute <scp>T<\/scp>hrombotic <scp>T<\/scp>hrombocytopenic 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