{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T01:05:25Z","timestamp":1790643925878,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,4,25]],"date-time":"2023-04-25T00:00:00Z","timestamp":1682380800000},"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":["Transfusion"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>There is a small but growing number of thrombotic thrombocytopenic purpura (TTP) cases attributed to immune checkpoint inhibitor therapy, with nivolumab and ipilimumab therapy being the most frequently described in the literature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design and Methods<\/jats:title><jats:p>This report evaluates the course of a patient with a history of metastatic adenocarcinoma of the lung who developed TTP following treatment with the PD\u20101 inhibitor Pembrolizumab. The patient was treated with six sessions of therapeutic plasma exchange and appeared to be in remission. Exacerbation occurred 4\u2009days later, and seven more sessions of plasma exchange were performed along with four total doses of Rituximab, and a steroid taper with monitoring of platelet counts and ADAMTS13 activity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>His platelet count recovered to a peak of 318,000 UL with an ADAMTS13 activity of 77% at the time of discharge. The patient has been following up regularly for outpatient testing with no TTP relapse as of the completion of this report.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>This is one of a few cases of Pembrolizumab\u2010associated TTP reported in the literature with successful complete remission following treatment. Plasma exchange in this setting may be an especially beneficial therapeutic intervention because of the removal of both the anti\u2010ADAMTS13 antibody as well as the immune system upregulating anti\u2010PDL1 monoclonal antibody with replacement of ADAMTS13 from donor plasma. Longer duration of plasma exchange and monitoring for normalization of ADAMTS13 levels in addition to platelet count before cessation of treatment may improve durable remission rates in this entity.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.17378","type":"journal-article","created":{"date-parts":[[2023,4,25]],"date-time":"2023-04-25T12:07:00Z","timestamp":1682424420000},"page":"1241-1245","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Durable remission of thrombotic thrombocytopenic purpura in the setting of pembrolizumab therapy"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4306-1754","authenticated-orcid":false,"given":"Michael","family":"Kozak","sequence":"first","affiliation":[{"name":"Pathology and Laboratory Medicine Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wesley","family":"Rubenstein","sequence":"additional","affiliation":[{"name":"Department of Clinical Pathology and Laboratory Medicine UC Davis  Davis California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derick","family":"Okwan\u2010Duodu","sequence":"additional","affiliation":[{"name":"Stanford Medicine  Stanford California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth","family":"Friedman","sequence":"additional","affiliation":[{"name":"Versiti Blood Research Institute  Milwaukee Wisconsin USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youram","family":"Nassir","sequence":"additional","affiliation":[{"name":"Hematology Oncology Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingrid","family":"Perez\u2010Alvarez","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.","family":"Gibb","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"Klapper","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8384-3227","authenticated-orcid":false,"given":"Samuel","family":"Pepkowitz","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine Cedars\u2010Sinai Medical Center  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,4,25]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41551-019-0360-0"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1501824"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/1078155219887212"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2017.00730"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjms.2021.10.024"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics12102559"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12883-022-02987-6"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2022-251966"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2021-246443"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2022.109015"},{"issue":"10","key":"e_1_2_6_12_1","article-title":"Thrombotic thrombocytopenic purpura induced by immune checkpoint Inhibitiors: a case report and review of the literature","volume":"12","author":"Ali Z","year":"2020","journal-title":"Cureus"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40425-017-0224-7"},{"key":"e_1_2_6_14_1","first-page":"1","article-title":"Refractory thrombotic thrombocytopenic purpura related to checkpoint inhibitor immunotherapy","volume":"61","author":"Lancelot M","year":"2020","journal-title":"Transfusion"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/2464619"},{"key":"e_1_2_6_16_1","unstructured":"vonAuer\u2010WegenerC SchmidtmannI BachL et al.Longitudinal assessment of ADAMTS13\u2010activity helps predict recurrence of immune thrombotic thrombocytopenic purpura (iTTP): results from the German TTP\u2010registry. 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