{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T09:13:46Z","timestamp":1792919626825,"version":"4.5.0"},"reference-count":105,"publisher":"American Society of Hematology","issue":"11","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,9,12]]},"abstract":"<jats:sec>\n                    <jats:title>Abstract<\/jats:title>\n                    <jats:p>In the 100 years since Eli Moschcowitz reported the first case of thrombotic thrombocytopenic purpura (TTP), there has been remarkable awareness and progress in the diagnosis and management of this rare blood disorder. This progress initially was the result of careful clinical observations followed by well thought-out therapeutic interventions, with dual goals of both improving outcomes and discerning the pathophysiology of TTP. The discovery of the ADAMTS13 protease set in motion the efforts to more accurately define the specific etiologies of thrombotic microangiopathies (TMAs) based on objective, scientific data rather than clinical characterizations alone. This accurate differentiation led to better and more revealing clinical trials and advancements in the treatment of TTP and other TMAs. Further advances followed and included improvements in immune-suppressive therapy and targeted therapies of immune-mediated TTP (iTTP; caplacizumab) and congenital TTP (cTTP; recombinant ADAMTS13). The longitudinal study of patients with TTP revealed the unexpected risk for long-term complications in both patients with iTTP and those with cTTP in remission. Ongoing studies aim to further understand the prevalence, mechanisms, and appropriate screening for these mood disorders, neurocognitive deficits, and cardiovascular complications that develop at remarkably high rates and are associated with a decreased life expectancy. These discoveries are a result of the collaborative efforts of investigators worldwide that have been fostered by the frequent interactions of investigators via the International TTP Working Group meetings and TMA workshops held regularly at international meetings. These efforts will support the rapid pace of discovery and improved understanding of this rare disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1182\/blood.2023022277","type":"journal-article","created":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T09:06:33Z","timestamp":1719997593000},"page":"1143-1152","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":24,"title":["Thrombotic thrombocytopenic purpura: 100 years of research on Moschcowitz syndrome"],"prefix":"10.1182","volume":"144","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8977-6910","authenticated-orcid":false,"given":"Spero R.","family":"Cataland","sequence":"first","affiliation":[{"name":"1Department of Medicine, Ohio State University, Columbus, OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Coppo","sequence":"additional","affiliation":[{"name":"2Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques, Service d'H\u00e9matologie, H\u00f4pital Saint-Antoine, Sorbonne Universit\u00e9, Assistance Publique\u2013H\u00f4pitaux de Paris, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Scully","sequence":"additional","affiliation":[{"name":"3Department of Clinical Haematology, University College London Hospitals NHS Foundation Trust, London, United Kingdom"},{"name":"4National Institute for Health Research University College London Hospital\/University College London Biomedical Research Centre, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4538-5154","authenticated-orcid":false,"given":"Bernhard","family":"L\u00e4mmle","sequence":"additional","affiliation":[{"name":"5Center for Thrombosis and Hemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Germany"},{"name":"6Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2024091512253883800_bib1","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 NY Pathol Soc"},{"issue":"21","key":"2024091512253883800_bib2","doi-asserted-by":"crossref","first-page":"2836","DOI":"10.1182\/blood-2016-10-709857","article-title":"Thrombotic thrombocytopenic purpura","volume":"129","author":"Joly","year":"2017","journal-title":"Blood"},{"issue":"3","key":"2024091512253883800_bib3","first-page":"536","article-title":"Thrombotic thrombocytopenic purpura: pathophysiology, diagnosis, and management","volume":"10","author":"Sukumar","year":"2021","journal-title":"J\u00a0Clin Med"},{"issue":"10","key":"2024091512253883800_bib4","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1182\/blood-2017-04-636431","article-title":"Pathophysiology of thrombotic thrombocytopenic purpura","volume":"130","author":"Sadler","year":"2017","journal-title":"Blood"},{"issue":"8","key":"2024091512253883800_bib5","doi-asserted-by":"crossref","first-page":"1864","DOI":"10.1111\/jth.15406","article-title":"The standard of care for immune thrombotic thrombocytopenic purpura today","volume":"19","author":"Zheng","year":"2021","journal-title":"J\u00a0Thromb Haemost"},{"issue":"6","key":"2024091512253883800_bib6","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1182\/blood.V2.6.542.542","article-title":"Thrombotic thrombocytopenic purpura; 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