{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T02:38:36Z","timestamp":1789785516396,"version":"4.0.1"},"reference-count":35,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,3,24]],"date-time":"2016-03-24T00:00:00Z","timestamp":1458777600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Acquired thrombotic thrombocytopenic purpura (\n                    <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>\n                    ) is a rare, life\u2010threatening condition caused by autoantibody\u2010mediated inhibition of\n                    <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                    13 (\n                    <jats:italic>a d<\/jats:italic>\n                    isintegrin\n                    <jats:italic>a<\/jats:italic>\n                    nd\n                    <jats:italic>m<\/jats:italic>\n                    etalloproteinase with\n                    <jats:italic>t<\/jats:italic>\n                    hrombo\n                    <jats:italic>s<\/jats:italic>\n                    pondin type\u20101 motif, 13). Therapeutic plasma exchange (\n                    <jats:styled-content style=\"fixed-case\">TPE<\/jats:styled-content>\n                    ) improves survival, but disease may be refractory despite therapy. Management and treatment response of refractory\n                    <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>\n                    is variable, with rituximab and other immunosuppression often being used. Case reports have suggested a benefit of the proteasome inhibitor, bortezomib, possibly due to elimination of the autoreactive plasma cells producing anti\u2010\n                    <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                    13 antibodies. We evaluated the effect of bortezomib in a series of primary refractory\n                    <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>\n                    patients unresponsive to intensive therapy. Bortezomib\u2010treated patients were identified from consecutive cases managed at two\n                    <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content>\n                    referral centres. Demographic and clinical data were extracted from hospital records.\n                    <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                    13 activity was measured using a fluorescence resonance energy transfer\n                    <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                    73 assay, and anti\u2010\n                    <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                    13 IgG using enzyme\u2010linked immunosorbent asssay. We identified six bortezomib\u2010treated patients out of 51 consecutive cases of acute, acquired\n                    <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>\n                    . All patients received\n                    <jats:styled-content style=\"fixed-case\">TPE<\/jats:styled-content>\n                    , methylprednisolone and rituximab. Five of the six achieved complete remission with bortezomib, and one died of cardiac arrest due to underlying disease. No treatment\u2010related adverse events were observed. Mean follow\u2010up time after hospital discharge was 17\u00a0months (range: 3\u201333). Bortezomib appears effective in the treatment of a subgroup of cases with severe, refractory\n                    <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>\n                    . Prospective trials are required to further investigate this effect.\n                  <\/jats:p>","DOI":"10.1111\/bjh.13993","type":"journal-article","created":{"date-parts":[[2016,3,24]],"date-time":"2016-03-24T06:44:13Z","timestamp":1458801853000},"page":"779-785","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":76,"title":["Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura"],"prefix":"10.1111","volume":"173","author":[{"given":"Christopher J.","family":"Patriquin","sequence":"first","affiliation":[{"name":"Division of Hematology &amp; Thromboembolism McMaster University Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari R.","family":"Thomas","sequence":"additional","affiliation":[{"name":"Department of Haematology University College London Hospital Cardiometabolic Programme\u2010NIHR UCLH\/UCL BRC London 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