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Given its heterogeneous clinical presentation, the phenotype of TTP during pregnancy and its management have not been well documented.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We report here a 25-year-old woman, G1P0 at 36\u2009weeks gestation, who developed severe thrombocytopenia and anemia. She was performed an emergent caesarean section 1 day after admission because of multiple organ failure. As ADAMTS 13 enzyme activity of the patient was 0% and antibodies were identified by enzyme-linked immunosorbent assay, she was diagnosed as acquired thrombotic thrombocytopenic purpura (aTTP). Furthermore, asymptomatic primary Sj\u00f6gren\u2019s syndrome was incidentally diagnosed on screening. After treatment with rituximab in addition to PEX and steroids, the activity of the ADAMTS 13 enzyme increased significantly from 0 to 100%.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>To the best of our knowledge, this is the first case report of concomitant TTP and asymptomatic Sj\u00f6gren\u2019s syndrome in a pregnant woman. It highlights the association between pregnancy, autoimmune disease, and TTP. It also emphasizes the importance of an enzyme-linked immunosorbent assay in the diagnosis and rituximab in the treatment of patients with acquired TTP.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12884-021-04167-9","type":"journal-article","created":{"date-parts":[[2021,12,3]],"date-time":"2021-12-03T10:06:29Z","timestamp":1638525989000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sj\u00f6gren\u2019s syndrome and non-neutralising antibodies to ADAMTS13: a case report"],"prefix":"10.1186","volume":"21","author":[{"given":"Lu","family":"Zhou","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miao","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Su","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaofan","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yizhi","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hui","family":"Mu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jie","family":"Yin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haiyan","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Weidong","family":"Pan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5011-6357","authenticated-orcid":false,"given":"Min","family":"Su","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,12,3]]},"reference":[{"issue":"10","key":"4167_CR1","doi-asserted-by":"publisher","first-page":"2453","DOI":"10.1111\/trf.14804","volume":"58","author":"W Plautz","year":"2018","unstructured":"Plautz W, Raval J, Dyer M, Rollins-Raval M. 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Our study was submitted to and approved by our institutional ethics committee. And the name of the ethics committee is Ethics Committee of Affiliated Hospital of Nantong University.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"<!--Emphasis Type='Bold' removed-->Ethics approval and consent to participate"}},{"value":"The patient who is the subject of this report provided written informed consent for the details of his case to be published.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"804"}}