{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T02:44:54Z","timestamp":1791081894411,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2014,9,11]],"date-time":"2014-09-11T00:00:00Z","timestamp":1410393600000},"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":[[2015,2]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Transplant\u2010associated thrombotic microangiopathy (<jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>) is a multifactorial disorder, which occurs as a result of treatment\u2010related endothelial injury and underlying disease process after hematopoietic stem cell transplantation (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>). The reported incidence of <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> after <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> is 0% to 74% and has shown to be associated with mortality rate of up to 100%. <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> is often diagnosed late in the disease progression, and therapeutic plasma exchange (<jats:styled-content style=\"fixed-case\">TPE<\/jats:styled-content>) has not been shown to produce a high response rate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design and Methods<\/jats:title><jats:p>All <jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>nglish\u2010language articles describing pharmacologic treatments for <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> were identified using <jats:styled-content style=\"fixed-case\">O<\/jats:styled-content>vid in the <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>edline database (1966\u2010<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>ay 2014). Search was limited to the <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> population.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Approximately 50% to 63% of patients with <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> respond to withdrawal of the offending agent (calcineurin inhibitors) and <jats:styled-content style=\"fixed-case\">TPE<\/jats:styled-content>, and many will require additional treatment to better control the disease. Unfortunately, there is no established treatment strategy for <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>. A number of pharmacologic agents that have been explored for the treatment of <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> include rituximab, vincristine, defibrotide, pravastatin, and eculizumab. The overall response rates of these agents were similar (69%\u201080%); however, the differences in the treatment costs vary significantly between these agents. Defibrotide is an investigational agent in the <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>nited <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>tates; therefore, it is not readily available for use.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Larger studies are warranted to validate the role of these pharmacologic agents in <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> as upfront therapy and in <jats:styled-content style=\"fixed-case\">TPE<\/jats:styled-content>\u2010refractory patients. Recently suggested predictive biomarkers for <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>, such as neutrophil extracellular traps and circulating endothelial cells, deserve more attention in future studies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.12859","type":"journal-article","created":{"date-parts":[[2014,9,11]],"date-time":"2014-09-11T08:54:05Z","timestamp":1410425645000},"page":"452-458","source":"Crossref","is-referenced-by-count":51,"title":["Hematopoietic stem cell transplant\u2013associated thrombotic microangiopathy: review of pharmacologic treatment options"],"prefix":"10.1111","volume":"55","author":[{"given":"Sara S.","family":"Kim","sequence":"first","affiliation":[{"name":"Department of Pharmacy The Mount Sinai Hospital  New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monank","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Pharmacy West Penn Hospital Allegheny Health Network  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kendra","family":"Yum","sequence":"additional","affiliation":[{"name":"Department of Pharmacy The Mount Sinai Hospital  New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alla","family":"Keyzner","sequence":"additional","affiliation":[{"name":"Blood and Marrow Transplantation Program Division of Hematology\/Oncology The Mount Sinai Hospital  New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,9,11]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.2165\/00003495-200969020-00004"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-321315"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1473-0502(02)00020-4"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705160"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e3181f24e8d"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-05-501445"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e31829807aa"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2013.09.005"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2005.06.001"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2004.00700.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002770050012"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-8588(05)70348-8"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1700889"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/jca.10008"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.10699"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2012.03776.x"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705241"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/1744-9987.12015"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/jca.2920060104"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.TP.0000141363.81600.0D"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1703414"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2007.06588.x"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12202"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2013.12.565"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1744-9987.2011.00978.x"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2010.01408.x"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705657"},{"key":"e_1_2_7_29_1","first-page":"72","article-title":"Successful treatment of transplant\u2010associated microangiopathy with rituximab","volume":"122","author":"Marr H","year":"2009","journal-title":"N Z Med J"},{"key":"e_1_2_7_30_1","unstructured":"Rituxan\u00ae [package insert]. 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