{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:05:37Z","timestamp":1790424337754,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,10,9]],"date-time":"2013-10-09T00:00:00Z","timestamp":1381276800000},"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":[[2014,5]]},"abstract":"\n Background<\/jats:title>\n \n Thrombotic thrombocytopenic purpura (\n TTP<\/jats:styled-content>\n ) is a life\u2010threatening disease resulting in systemic microvascular thrombosis. The disease is caused by excessive platelet (PLT) adhesion to ultra\u2010large (UL) von\n W<\/jats:styled-content>\n illebrand factor (\n VWF<\/jats:styled-content>\n ) multimers inadequately cleaved by the processing enzyme\n ADAMTS<\/jats:styled-content>\n \u201013. While many cases respond to plasma exchange performed with or without concurrent corticosteroids, treatment of the 10% to 20% of patients with refractory disease is difficult. Experimental studies demonstrating that\n \n N<\/jats:styled-content>\n <\/jats:italic>\n \u2010acetylcysteine (\n NAC<\/jats:styled-content>\n ) inhibits\n PLT<\/jats:styled-content>\n binding to endothelial cell\u2013secreted and anchored UL\n VWF<\/jats:styled-content>\n multimers suggest that\n NAC<\/jats:styled-content>\n may be useful in the treatment of\n TTP<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Case Report<\/jats:title>\n \n A 44\u2010year\u2010old woman presented with malaise, confusion, chest and abdominal pain, and transient visual loss. Laboratory results and peripheral blood smear were consistent with\n TTP<\/jats:styled-content>\n . The patient was begun on plasma exchange and corticosteroid treatment, but after 10 days the PLT count was still less than 10.0\u2009\u00d7\u200910\n 9<\/jats:sup>\n \/L and she developed a fever. Rituximab was initiated, but the patient's condition worsened and she became comatose. Antibiotics were initiated, but cultures remained sterile. After 3\u2009days of coma and further clinical deterioration, treatment with\n N<\/jats:styled-content>\n AC<\/jats:styled-content>\n was begun. The patient received a loading dose of 150\u2009mg\/kg\n N<\/jats:styled-content>\n AC<\/jats:styled-content>\n intravenously (\n IV<\/jats:styled-content>\n ) over 1 hour. Within 18\u2009hours the patient awakened abruptly and began communicating with medical personnel. Plasma exchange, corticosteroids, rituximab, and\n N<\/jats:styled-content>\n AC<\/jats:styled-content>\n infusion (150\u2009mg\/kg\n IV<\/jats:styled-content>\n over 17\u2009hr daily\u2009\u00d7\u200910 days) were continued and by\n D<\/jats:styled-content>\n ay 17 the\n PLT<\/jats:styled-content>\n count was more than 50\u2009\u00d7\u200910\n 9<\/jats:sup>\n \/L. The patient fully recovered and was discharged on\n D<\/jats:styled-content>\n ay 31.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n This is the first complete report of a\n TTP<\/jats:styled-content>\n patient treated with\n NAC<\/jats:styled-content>\n .\n NAC<\/jats:styled-content>\n was a safe and effective supplementary treatment for refractory\n TTP<\/jats:styled-content>\n in this patient.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/trf.12440","type":"journal-article","created":{"date-parts":[[2013,10,11]],"date-time":"2013-10-11T22:33:44Z","timestamp":1381530824000},"page":"1221-1224","source":"Crossref","is-referenced-by-count":56,"title":["Treatment of refractory thrombotic thrombocytopenic purpura with\n \n N<\/scp>\n <\/i>\n \u2010acetylcysteine: a case report"],"prefix":"10.1111","volume":"54","author":[{"given":"Gloria W.","family":"Li","sequence":"first","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"},{"name":"The Hematology Service Ben Taub General Hospital Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siayareh","family":"Rambally","sequence":"additional","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"},{"name":"The Hematology Service Ben Taub General Hospital Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasmine","family":"Kamboj","sequence":"additional","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"},{"name":"The Hematology Service Ben Taub General Hospital Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean","family":"Reilly","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Harris Health Systems Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel L.","family":"Moake","sequence":"additional","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark M.","family":"Udden","sequence":"additional","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"},{"name":"The Hematology Service Ben Taub General Hospital Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martha P.","family":"Mims","sequence":"additional","affiliation":[{"name":"Department of Medicine Section of Hematology Oncology Baylor College of Medicine Houston Texas"},{"name":"The Hematology Service Ben Taub General Hospital Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,10,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMcp053024"},{"key":"e_1_2_6_3_1","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1182\/asheducation.V2012.1.604.3798564","article-title":"Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes","volume":"2012","author":"George JN","year":"2012","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199811263392202"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199811263392203"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199108083250604"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-07-271445"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI41062"},{"key":"e_1_2_6_9_1","first-page":"114","article-title":"Clinical applications of N\u2010acetylcysteine","volume":"3","author":"Kelly GS","year":"1998","journal-title":"Altern Med Rev"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-009-0877-5"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2007.01530.x"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-341131"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-012-0723-9"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1300950"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2012.09084.x"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12387"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.3109\/00365519409088566"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/CCM.0b013e31819ffed4"},{"key":"e_1_2_6_19_1","unstructured":"ClinicalTrials.gov. 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