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Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates. In more severe patients, salvage strategies may include twice-daily TPE, pulses of cyclophosphamide, vincristine, as well as splenectomy in more desperate cases. In this life-threatening disease, relapse prevention represents a major goal. Persistent severe acquired ADAMTS13 deficiency in patients who are otherwise in remission is associated with a high risk of relapse and preemptive treatment with rituximab may be considered in this context. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, ideally international, clinical trials. Promising agents under evaluation include N-acetylcysteine, bortezomib, recombinant ADAMTS13, and inhibitors of the glycoprotein-Ib\/IX-von Willebrand factor axis.<\/jats:p>","DOI":"10.1182\/asheducation-2015.1.637","type":"journal-article","created":{"date-parts":[[2016,9,15]],"date-time":"2016-09-15T15:23:25Z","timestamp":1473953005000},"page":"637-643","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":51,"title":["Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange"],"prefix":"10.1182","volume":"2015","author":[{"given":"Paul","family":"Coppo","sequence":"first","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques, AP-HP, Paris, France;"},{"name":"Service d'h\u00e9matologie, H\u00f4pital Saint Antoine, Paris, France;"},{"name":"Inserm U1170, Institut Gustave Roussy, Villejuif, France;"},{"name":"Universit\u00e9 Pierre et Marie Curie, Paris, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Froissart","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques, AP-HP, Paris, France;"},{"name":"Service de m\u00e9decine interne, CHI Cr\u00e9teil, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"issue":"3 Pt 2","key":"2019111704475200600_B1","doi-asserted-by":"crossref","first-page":"e163","DOI":"10.1016\/j.lpm.2011.10.024","article-title":"Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura","volume":"41","author":"Coppo","year":"2012","journal-title":"Presse Med"},{"issue":"6","key":"2019111704475200600_B2","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1056\/NEJM199108083250604","article-title":"Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura: Canadian Apheresis Study Group","volume":"325","author":"Rock","year":"1991","journal-title":"N Engl J Med"},{"issue":"1","key":"2019111704475200600_B3","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1182\/blood-2008-02-078170","article-title":"Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura","volume":"112","author":"Sadler","year":"2008","journal-title":"Blood"},{"issue":"6","key":"2019111704475200600_B4","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1056\/NEJM199108083250605","article-title":"Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. 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