{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T00:17:27Z","timestamp":1793146647460,"version":"4.5.0"},"reference-count":88,"publisher":"Elsevier BV","issue":"12","license":[{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["clinicalkey.es","elsevier.es","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Medicina Cl\u00ednica"],"published-print":{"date-parts":[[2022,6]]},"DOI":"10.1016\/j.medcli.2021.03.040","type":"journal-article","created":{"date-parts":[[2021,7,12]],"date-time":"2021-07-12T22:26:54Z","timestamp":1626128814000},"page":"630.e1-630.e14","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":17,"title":["Recomendaciones para el abordaje cl\u00ednico de pacientes con p\u00farpura trombocitop\u00e9nica tromb\u00f3tica"],"prefix":"10.1016","volume":"158","author":[{"given":"Mar\u00eda Eva","family":"Mingot Castellano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Pascual Izquierdo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ataulfo","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurora","family":"Viejo Llorente","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Valcarcel Ferreiras","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Sebasti\u00e1n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faustino","family":"Garc\u00eda Candel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9ctor","family":"Sarmiento Palao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00e9s","family":"G\u00f3mez Segu\u00ed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier","family":"de la Rubia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joan","family":"Cid","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Mart\u00ednez Nieto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis","family":"Hern\u00e1ndez Mateo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Goterris Viciedo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Fidalgo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramon","family":"Salinas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julio","family":"del Rio-Garma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.medcli.2021.03.040_bib0445","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1111\/jth.13571","article-title":"Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies","volume":"15","author":"Scully","year":"2017","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0450","doi-asserted-by":"crossref","DOI":"10.1182\/blood.2020009150","article-title":"Redefining outcomes in immune TTP: an international working group consensus report","author":"Cuker","year":"2021","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0455","article-title":"Incidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura","volume":"134","author":"Pascual Izquierdo","year":"2021","journal-title":"J Clin Apher."},{"key":"10.1016\/j.medcli.2021.03.040_bib0460","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."},{"key":"10.1016\/j.medcli.2021.03.040_bib0465","first-page":"331","article-title":"Diagnostic and therapeutic guidelines of thrombotic microangiopathies of the Spanish Apheresis Group","volume":"144","author":"Contreras","year":"2015","journal-title":"Med Clin (Barc)."},{"key":"10.1016\/j.medcli.2021.03.040_bib0470","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1111\/j.1365-2141.2012.09167.x","article-title":"Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies","volume":"158","author":"Scully","year":"2012","journal-title":"Br J Haematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0475","doi-asserted-by":"crossref","first-page":"e89","DOI":"10.1016\/j.amjmed.2020.06.020","article-title":"Cardiovascular complications and their association with mortality in patients with thrombotic thrombocytopenic purpura","volume":"134","author":"Balasubramaniyam","year":"2021","journal-title":"Am J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0480","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1016\/j.medcli.2010.02.011","article-title":"Thrombotic thrombocytopenic purpura","volume":"136","author":"De La Rubia","year":"2011","journal-title":"Med Clin (Barc)."},{"key":"10.1016\/j.medcli.2021.03.040_bib0485","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1002\/rth2.12160","article-title":"Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine","volume":"3","author":"Coppo","year":"2019","journal-title":"Res Pract Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0490","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","volume":"325","author":"Rock","year":"1991","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0495","doi-asserted-by":"crossref","first-page":"E644","DOI":"10.1002\/ajh.24885","article-title":"Diagnosis of thrombotic thrombocytopenic purpura among patients with ADAMTS13 Activity 10%-20%","volume":"92","author":"Ayanambakkam","year":"2017","journal-title":"Am J Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0500","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.1055\/s-0039-1696718","article-title":"Evaluation of a New, Rapid Fully Automated Assay for the Measurement of ADAMTS13 Activity","volume":"119","author":"Valsecchi","year":"2019","journal-title":"Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0505","doi-asserted-by":"crossref","first-page":"1686","DOI":"10.1111\/jth.14815","article-title":"A multi-center evaluation of TECHNOSCREEN\u00ae ADAMTS-13 activity assay as a screening tool for detecting deficiency of ADAMTS-13","volume":"18","author":"Moore","year":"2020","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0510","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1111\/ijlh.13414","article-title":"Multicentric evaluation of the new HemosIL Acustar\u00ae chemiluminescence ADAMTS13 activity assay","volume":"43","author":"Pascual","year":"2021","journal-title":"Int J Lab Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0515","doi-asserted-by":"crossref","first-page":"2486","DOI":"10.1111\/jth.15006","article-title":"ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura","volume":"18","author":"Zheng","year":"2020","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0520","doi-asserted-by":"crossref","first-page":"e10208","DOI":"10.1371\/journal.pone.0010208","article-title":"Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: The French TMA reference center experience","volume":"5","author":"Coppo","year":"2010","journal-title":"PLoS One."},{"key":"10.1016\/j.medcli.2021.03.040_bib0525","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1515\/cclm-2017-0437","article-title":"Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies","volume":"56","author":"Nieto","year":"2018","journal-title":"Clin Chem Lab Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0530","doi-asserted-by":"crossref","first-page":"e157","DOI":"10.1016\/S2352-3026(17)30026-1","article-title":"Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study","volume":"4","author":"Bendapudi","year":"2017","journal-title":"Lancet Haematol."},{"issue":"Suppl. 1","key":"10.1016\/j.medcli.2021.03.040_bib0535","doi-asserted-by":"crossref","first-page":"2440","DOI":"10.1182\/blood-2018-99-111416","article-title":"Plasmic score in thrombotic microangiopathy may not be discriminatory in many instances","volume":"132","author":"Reddy","year":"2018","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0540","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1056\/NEJMoa1806311","article-title":"Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura","volume":"380","author":"Scully","year":"2019","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0545","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1111\/jth.12790","article-title":"Cardiac troponin-i on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. experience of the french thrombotic microangiopathies reference center","volume":"13","author":"Benhamou","year":"2015","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0550","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1182\/blood-2016-12-758656","article-title":"Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura","volume":"130","author":"Alwan","year":"2017","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0555","doi-asserted-by":"crossref","first-page":"1526","DOI":"10.1111\/jth.13016","article-title":"Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity: reply","volume":"13","author":"Zafrani","year":"2015","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0560","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1056\/NEJMoa1505533","article-title":"Caplacizumab for acquired thrombotic thrombocytopenic purpura","volume":"374","author":"Peyvandi","year":"2016","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0565","unstructured":"Agencia Europea del Medicamento (EMA) Ficha t\u00e9cnica o resumen de las caracter\u00edsticas del producto: Caplacizumab. 2018"},{"key":"10.1016\/j.medcli.2021.03.040_bib0570","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1111\/j.1537-2995.2006.00959.x","article-title":"A randomized, controlled Phase III trial of therapeutic plasma exchange with fresh frozen plasma (FFP) prepared with amotosalen and ultraviolet A light compared to untreated FFP in thrombotic thrombocytopenic purpura","volume":"46","author":"Mintz","year":"2006","journal-title":"Transfusion."},{"key":"10.1016\/j.medcli.2021.03.040_bib0575","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1111\/j.1365-2141.2008.07292.x","article-title":"Methylene blue-photoinactivated plasma versus quarantine fresh frozen plasma in thrombotic thrombocytopenic purpura: A multicentric, prospective cohort study","volume":"143","author":"Del R\u00edo-Garma","year":"2008","journal-title":"Br J Haematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0580","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1002\/jca.21288","article-title":"The use of 50% albumin\/plasma replacement fluid in therapeutic plasma exchange for thrombotic thrombocytopenic purpura","volume":"28","author":"O\u2019Brien","year":"2013","journal-title":"J Clin Apher."},{"key":"10.1016\/j.medcli.2021.03.040_bib0585","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1002\/jca.21475","article-title":"Two approaches to the clinical dilemma of treating TTP with therapeutic plasma exchange in patients with a history of anaphylactic reactions to plasma","volume":"32","author":"Sidhu","year":"2017","journal-title":"J Clin Apher."},{"key":"10.1016\/j.medcli.2021.03.040_bib0590","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1002\/jca.21771","article-title":"Efficacy of albumin and compounded plasma for plasma exchange in acquired thrombotic thrombocytopenic purpura","volume":"35","author":"Hou","year":"2020","journal-title":"J Clin Apher."},{"key":"10.1016\/j.medcli.2021.03.040_bib0595","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1111\/trf.15901","article-title":"Therapeutic. plasma exchange taper does not decrease exacerbations in immune thrombotic. thrombocytopenic purpura patients","volume":"60","author":"Raval","year":"2020","journal-title":"Transfusion."},{"key":"10.1016\/j.medcli.2021.03.040_bib0600","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1056\/NEJM199108083250605","article-title":"Improved survival in thrombotic thrombocytopenic purpura\u2013hemolytic uremic syndrome: Clinical experience in 108 patients","volume":"325","author":"Bell","year":"1991","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0605","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1007\/s00277-009-0877-5","article-title":"High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: A randomized study","volume":"89","author":"Balduini","year":"2010","journal-title":"Ann Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0610","doi-asserted-by":"crossref","first-page":"1746","DOI":"10.1182\/blood-2011-03-341131","article-title":"A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura","volume":"118","author":"Scully","year":"2011","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0615","doi-asserted-by":"crossref","first-page":"3092","DOI":"10.1182\/blood-2016-03-703827","article-title":"Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura","volume":"127","author":"Page","year":"2016","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0620","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1097\/CCM.0b013e31822e9d66","article-title":"Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center","volume":"40","author":"Froissart","year":"2012","journal-title":"Crit Care Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0625","doi-asserted-by":"crossref","first-page":"2496","DOI":"10.1111\/jth.15010","article-title":"ISTH guidelines for treatment of thrombotic thrombocytopenic purpura","volume":"18","author":"Zheng","year":"2020","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0635","doi-asserted-by":"crossref","first-page":"2137","DOI":"10.1182\/bloodadvances.2020001834","article-title":"Caplacizumab prevents refractoriness and mortality in acquired thrombotic thrombocytopenic purpura: integrated analysis","volume":"5","author":"Peyvandi","year":"2021","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0630","doi-asserted-by":"crossref","first-page":"3085","DOI":"10.1182\/bloodadvances.2020001973","article-title":"Real- world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura","volume":"4","author":"Volker","year":"2020","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0640","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1182\/blood.2020008021","article-title":"A regimen with caplacizumab, immunosuppression and plasma exchange prevents unfavorable outcomes in immune-mediated TTP","volume":"137","author":"Coppo","year":"2021","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0645","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.1182\/blood.2020007599","article-title":"Real-world evidence of caplacizumab use in the management of acute TTP","volume":"137","author":"Dutt","year":"2021","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0650","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1111\/jth.15194","article-title":"Should all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab?","volume":"19","author":"Picod","year":"2021","journal-title":"J Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0655","doi-asserted-by":"crossref","first-page":"1030110","DOI":"10.1016\/j.transci.2020.103011","article-title":"Successful management of three patients with autoimmune thrombotic thrombocytopenic purpura with paradigm-changing therapy: Caplacizumab, steroids, plasma exchange, rituximab, and intravenous immunoglobulins (CASPERI)","volume":"60","author":"Cid","year":"2021","journal-title":"Transfus Apher Sci."},{"key":"10.1016\/j.medcli.2021.03.040_bib0660","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1182\/blood.2020006052","article-title":"Cost effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura","volume":"137","author":"Goshua","year":"2021","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0665","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1182\/blood-2016-09-738856","article-title":"N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura","volume":"129","author":"Tersteeg","year":"2017","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0670","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1111\/j.1537-2995.2008.02082.x","article-title":"Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura","volume":"49","author":"Swisher","year":"2009","journal-title":"Transfusion."},{"key":"10.1016\/j.medcli.2021.03.040_bib0675","unstructured":"Orphanet. SOAR HI: Estudio de fase II, multic\u00e9ntrico, aleatorizado, con doble enmascaramiento, controlado con placebo en pacientes con p\u00farpura trombocitop\u00e9nica tromb\u00f3tica adquirida PTTa para evaluar la farmacocin\u00e9tica, la seguridad y la eficacia de rADAMT s. f."},{"key":"10.1016\/j.medcli.2021.03.040_bib0680","doi-asserted-by":"crossref","first-page":"2055","DOI":"10.1182\/blood-2017-06-788026","article-title":"Recombinant ADAMTS-13: First-in-human pharmacokinetics and safety in congenital thrombotic thrombocytopenic purpura","volume":"130","author":"Scully","year":"2017","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0685","doi-asserted-by":"crossref","first-page":"1751","DOI":"10.1007\/s00277-016-2804-x","article-title":"Bortezomib therapy in patients with relapsed\/refractory acquired thrombotic thrombocytopenic purpura","volume":"95","author":"Eskazan","year":"2016","journal-title":"Ann Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0690","article-title":"Acquired TTP: Treatment of refractory or relapsed disease - UpToDate","author":"George","year":"2020","journal-title":"UpToDate."},{"key":"10.1016\/j.medcli.2021.03.040_bib0695","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1182\/asheducation-2018.1.539","article-title":"Beyond plasma exchange: Novel therapies for thrombotic thrombocytopenic purpura","volume":"2018","author":"Dane","year":"2018","journal-title":"Hematology Am Soc Hematol Educ Program."},{"key":"10.1016\/j.medcli.2021.03.040_bib0700","doi-asserted-by":"crossref","first-page":"3860","DOI":"10.1182\/blood-2014-11-551580","article-title":"How I treat refractory thrombotic thrombocytopenic purpura","volume":"125","author":"Sayani","year":"2015","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0705","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1159\/000505236","article-title":"Rituximab twice weekly for refractory thrombotic thrombocytopenic purpura in a critically ill patient with acute respiratory distress syndrome","volume":"13","author":"Azrieh","year":"2020","journal-title":"Case Rep Oncol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0710","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.mayocpiqo.2017.11.004","article-title":"Complement regulatory genetic mutations in the setting of autoimmune thrombotic thrombocytopenic purpura: a case series","volume":"2","author":"Atrash","year":"2018","journal-title":"Mayo Clin Proc Innov Qual Outcomes."},{"key":"10.1016\/j.medcli.2021.03.040_bib0715","doi-asserted-by":"crossref","first-page":"2436","DOI":"10.1111\/j.1537-2995.2012.03578.x","article-title":"Splenectomy and\/or cyclophosphamide as salvage therapies in thrombotic thrombocytopenic purpura: The French TMA Reference Center experience","volume":"52","author":"Beloncle","year":"2012","journal-title":"Transfusion."},{"key":"10.1016\/j.medcli.2021.03.040_bib0720","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1111\/bjh.13993","article-title":"Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura","volume":"173","author":"Patriquin","year":"2016","journal-title":"Br J Haematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0725","doi-asserted-by":"crossref","first-page":"2075","DOI":"10.1182\/bloodadvances.2017009308","article-title":"Cyclosporine or steroids as an adjunct to plasma exchange in the treatment of immune-mediated thrombotic thrombocytopenic purpura","volume":"1","author":"Cataland","year":"2017","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0730","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1002\/ajh.20678","article-title":"Outcomes in the treatment of thrombotic thrombocytopenic purpura with splenectomy: A retrospective cohort study","volume":"81","author":"Martinez Outschoorn","year":"2006","journal-title":"Am J Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0735","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/BF01784041","article-title":"The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpura","volume":"70","author":"Veltman","year":"1995","journal-title":"Ann Hematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0740","doi-asserted-by":"crossref","first-page":"4177","DOI":"10.1182\/bloodadvances.2019000939","article-title":"Longitudinal assessments of plasma ADAMTS13 biomarkers predict recurrence of immune thrombotic thrombocytopenic purpura","volume":"3","author":"Sui","year":"2019","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0745","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1182\/blood-2014-01-550244","article-title":"Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura","volume":"124","author":"Hie","year":"2014","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0750","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1182\/bloodadvances.2017008268","article-title":"Rituximab prophylaxis to prevent thrombotic thrombocytopenic purpura relapse: Outcome and evaluation of dosing regimens","volume":"1","author":"Westwood","year":"2017","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0755","doi-asserted-by":"crossref","first-page":"2143","DOI":"10.1182\/blood-2018-04-840090","article-title":"Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura","volume":"132","author":"Jestin","year":"2018","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0760","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1182\/bloodadvances.2019031039","article-title":"Predictors of relapse and efficacy of rituximab in immune thrombotic thrombocytopenic purpura","volume":"3","author":"Sun","year":"2019","journal-title":"Blood Adv."},{"key":"10.1016\/j.medcli.2021.03.040_bib0765","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1182\/asheducation-2018.1.548","article-title":"TTP: Long-term outcomes following recovery","volume":"2018","author":"George","year":"2018","journal-title":"Hematology Am Soc Hematol Educ Program."},{"key":"10.1016\/j.medcli.2021.03.040_bib0770","unstructured":"Montreal Cognitive Assessment [Evaluaci\u00f3n Cognitiva Montreal] (MoCA): Instrucciones para la administraci\u00f3n y computaci\u00f3n de resultados. Versi\u00f3n 12 de noviembre de 2004 (Z. Nasreddine MD. www.mocatest.org. Translated to Spanish, version September 22, 2006. s. f."},{"key":"10.1016\/j.medcli.2021.03.040_bib0775","first-page":"2000","author":"Alonso","year":"1996","journal-title":"Versi\u00f3n espa\u00f1ola de SF-36v2TM Health Survey \u00a9"},{"key":"10.1016\/j.medcli.2021.03.040_bib0780","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0212-6567(01)70406-4","article-title":"El EuroQol-5D: una alternativa sencilla para la medici\u00f3n de la calidad de vida relacionada con la salud en atenci\u00f3n primaria","volume":"28","author":"Herdman","year":"2001","journal-title":"Aten Primaria."},{"key":"10.1016\/j.medcli.2021.03.040_bib0785","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1111\/ejh.13107","article-title":"Pediatric thrombotic thrombocytopenic purpura","volume":"101","author":"Joly","year":"2018","journal-title":"Eur J Haematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0790","doi-asserted-by":"crossref","first-page":"e537","DOI":"10.1016\/S2352-3026(16)30125-9","article-title":"Child- onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy","volume":"3","author":"Joly","year":"2016","journal-title":"Lancet Haematol."},{"key":"10.1016\/j.medcli.2021.03.040_bib0795","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1097\/MOP.0b013e32835e7888","article-title":"Thrombotic thrombocytopenic purpura in children","volume":"25","author":"Loirat","year":"2013","journal-title":"Curr Opin Pediatr."},{"key":"10.1016\/j.medcli.2021.03.040_bib0800","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1002\/pbc.24612","article-title":"Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: Comparison of incidence, demographic and clinical features","volume":"60","author":"Reese","year":"2013","journal-title":"Pediatr Blood Cancer."},{"key":"10.1016\/j.medcli.2021.03.040_bib0805","unstructured":"Orkin S, Nathan D, Ginsburg D, Look AT, Fisher D, Lux S. Nathan and Oski's Hematology and Oncology of Infancy and Childhood, 2-Volume Set - 8th Edition. vol. 2. ed. 2014."},{"key":"10.1016\/j.medcli.2021.03.040_bib0810","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1186\/s12884-015-0557-5","article-title":"Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women: A difficult diagnosis and a treatable disease","volume":"15","author":"Delmas","year":"2015","journal-title":"BMC Pregnancy Childbirth."},{"key":"10.1016\/j.medcli.2021.03.040_bib0815","doi-asserted-by":"crossref","first-page":"100638","DOI":"10.1016\/j.blre.2019.100638","article-title":"Thrombocytopenia in pregnancy: Diagnosis and approach to management","volume":"40","author":"Pishko","year":"2020","journal-title":"Blood Rev."},{"key":"10.1016\/j.medcli.2021.03.040_bib0820","doi-asserted-by":"crossref","first-page":"1653","DOI":"10.1056\/NEJMra1813013","article-title":"Hereditary thrombotic thrombocytopenic purpura","volume":"381","author":"Hovinga","year":"2019","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0825","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1002\/rth2.12016","article-title":"Combined study of ADAMTS13 and complement genes in the diagnosis of thrombotic microangiopathies using next- generation sequencing","volume":"1","author":"Fidalgo","year":"2017","journal-title":"Res Pract Thromb Haemost."},{"key":"10.1016\/j.medcli.2021.03.040_bib0830","doi-asserted-by":"crossref","first-page":"2107","DOI":"10.3324\/haematol.2019.216796","article-title":"The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: Key findings at enrollment until 2017","volume":"104","author":"Van Dorland","year":"2019","journal-title":"Haematologica."},{"key":"10.1016\/j.medcli.2021.03.040_bib0835","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.1182\/blood-2018-11-884700","article-title":"Characterization and treatment of congenital thrombotic thrombocytopenic purpura","volume":"133","author":"Alwan","year":"2019","journal-title":"Blood."},{"key":"10.1016\/j.medcli.2021.03.040_bib0840","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: A joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0845","unstructured":"About hTTP. Upshaw Schulman syndrome. Hereditary TTP Registry. Disponible en https:\/\/ttpregistry.net\/about-http\/"},{"key":"10.1016\/j.medcli.2021.03.040_bib0850","unstructured":"REPTT. Disponible en https:\/\/reptt.gesdat.es\/"},{"key":"10.1016\/j.medcli.2021.03.040_bib0855","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1080\/0886022X.2018.1427110","article-title":"Evaluation of health-related quality of life in hemolytic uraemic syndrome patients treated with eculizumab: a systematic evaluation on basis of EMPRO","volume":"40","author":"Mukherjee","year":"2018","journal-title":"Ren Fail."},{"key":"10.1016\/j.medcli.2021.03.040_bib0860","doi-asserted-by":"crossref","first-page":"770","DOI":"10.4067\/s0034-98872018000600770","article-title":"S\u00edndrome hemol\u00edtico ur\u00e9mico at\u00edpico","volume":"146","author":"Sep\u00falveda","year":"2018","journal-title":"Rev Med Chile."},{"key":"10.1016\/j.medcli.2021.03.040_bib0865","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1038\/ki.2014.423","article-title":"Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies","volume":"87","author":"Licht","year":"2015","journal-title":"Kidney Int."},{"key":"10.1016\/j.medcli.2021.03.040_bib0870","doi-asserted-by":"crossref","first-page":"1377","DOI":"10.1056\/NEJMc1308826","article-title":"Eculizumab in atypical hemolytic\u2013uremic syndrome","volume":"369","author":"Legendre","year":"2013","journal-title":"N Engl J Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0875","doi-asserted-by":"crossref","first-page":"181","DOI":"10.2147\/JBM.S102235","article-title":"Hematopoietic cell transplantation-associated thrombotic microangiopathy: A review of pathophysiology, diagnosis, and treatment","volume":"7","author":"Rosenthal","year":"2016","journal-title":"J Blood Med."},{"key":"10.1016\/j.medcli.2021.03.040_bib0880","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1055\/s-0039-1687893","article-title":"Thrombotic microangiopathy in cancer","volume":"45","author":"Weitz","year":"2019","journal-title":"Semin Thromb Hemost."}],"container-title":["Medicina Cl\u00ednica"],"original-title":[],"language":"es","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0025775321003328?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0025775321003328?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,12,29]],"date-time":"2022-12-29T19:51:33Z","timestamp":1672343493000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0025775321003328"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6]]},"references-count":88,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022,6]]}},"alternative-id":["S0025775321003328"],"URL":"https:\/\/doi.org\/10.1016\/j.medcli.2021.03.040","relation":{},"ISSN":["0025-7753"],"issn-type":[{"value":"0025-7753","type":"print"}],"subject":[],"published":{"date-parts":[[2022,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Recomendaciones para el abordaje cl\u00ednico de pacientes con p\u00farpura trombocitop\u00e9nica tromb\u00f3tica","name":"articletitle","label":"Article Title"},{"value":"Medicina Cl\u00ednica","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.medcli.2021.03.040","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 Elsevier Espa\u00f1a, S.L.U. All rights reserved.","name":"copyright","label":"Copyright"}]}}