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A paucity of studies guiding severely refractory TTP makes management arbitrary and individualised, highlighting the value of isolated reports. We present an extremely rare case of primary refractory TTP with an insufficient platelet response to numerous types of treatments, including emerging therapies such as caplacizumab, on the background of repeated PEX and immunosuppressive therapies.<\/jats:p>","DOI":"10.1136\/bcr-2019-234091","type":"journal-article","created":{"date-parts":[[2020,6,12]],"date-time":"2020-06-12T05:55:19Z","timestamp":1591941319000},"page":"e234091","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":12,"title":["Severe primary refractory thrombotic thrombocytopenic purpura (TTP) in the post plasma exchange (PEX) and rituximab era"],"prefix":"10.1136","volume":"13","author":[{"given":"Sowmya","family":"Goranta","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, Michigan, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5239-9533","authenticated-orcid":false,"given":"Smit S","family":"Deliwala","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, Michigan, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8637-7292","authenticated-orcid":false,"given":"Tarek","family":"Haykal","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, Michigan, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ghassan","family":"Bachuwa","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, Michigan, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2020,6,11]]},"reference":[{"key":"2025082712303176000_13.6.e234091.1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1962.tb06541.x"},{"key":"2025082712303176000_13.6.e234091.2","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1016\/j.mayocp.2016.05.015","article-title":"Thrombotic microangiopathy care pathway: a consensus statement for the Mayo clinic complement alternative Pathway-Thrombotic microangiopathy (CAP-TMA) Disease-Oriented group","volume":"91","author":"RS","year":"2016","journal-title":"Mayo Clinic Proceedings [Internet]"},{"key":"2025082712303176000_13.6.e234091.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1312353"},{"key":"2025082712303176000_13.6.e234091.4","doi-asserted-by":"crossref","first-page":"782","DOI":"10.12659\/AJCR.898955","article-title":"Systemic lupus erythematosus presenting as refractory thrombotic thrombocytopenic purpura: a diagnostic and management challenge. 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