{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T20:40:03Z","timestamp":1756327203883,"version":"3.44.0"},"reference-count":17,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,7,23]]},"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:p>Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15\u2009mL\/kg body weight and continues to be in remission.<\/jats:p>","DOI":"10.1136\/bcr-2020-239901","type":"journal-article","created":{"date-parts":[[2021,8,11]],"date-time":"2021-08-11T13:00:27Z","timestamp":1628686827000},"page":"e239901","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy"],"prefix":"10.1136","volume":"14","author":[{"given":"Faheema","family":"Hasan","sequence":"first","affiliation":[{"name":"Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anshul","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dinesh","family":"Chandra","sequence":"additional","affiliation":[{"name":"Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soniya","family":"Nityanand","sequence":"additional","affiliation":[{"name":"Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,8,11]]},"reference":[{"key":"2025082713263143000_14.8.e239901.1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-10-709857"},{"key":"2025082713263143000_14.8.e239901.2","first-page":"21","article-title":"Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease","volume":"24","author":"Moschcowitz","year":"1924","journal-title":"Proc New York Pathol Soc"},{"key":"2025082713263143000_14.8.e239901.3","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1182\/blood.V16.1.943.943","article-title":"Studies on thrombopoiesis. 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