{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T01:24:27Z","timestamp":1789176267571,"version":"build-2803163510"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T00:00:00Z","timestamp":1718928000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Evans syndrome (ES) is rare and mostly treated on a \u201ccase\u2010by\u2010case\u201d basis and no guidelines are available. With the aim of assessing disease awareness and current management of adult ES, a structured survey was administered to 64 clinicians from 50 Italian participating centers. Clinicians had to be involved in the management of autoimmune cytopenias and were enrolled into the ITP\u2010NET initiative. The survey included domains on epidemiology, diagnosis, and therapy of ES and was designed to capture current practice and suggested work\u2010up and management. Thirty clinicians who had followed a median of 5 patients (1\u201345)\/15\u2009years responded. The combination of AIHA plus ITP was more common than the ITP\/AIHA with neutropenia (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001) and 25% of patients had an associated condition, including lymphoproliferative syndromes, autoimmune diseases, or primary immunodeficiencies. The agreement of clinicians for each diagnostic test is depicted (i.e., 100% for blood count and DAT; only 40% for anti\u2010platelets and anti\u2010neutrophils; 77% for bone marrow evaluation). Most clinicians reported that ES requires a specific approach compared to isolated autoimmune cytopenias, due to either a more complex pathogenesis and a higher risk of relapse and thrombotic and infectious complications. The heterogeneity of treatment choices among different physicians suggests the need for broader harmonization.<\/jats:p>","DOI":"10.1111\/ejh.14256","type":"journal-article","created":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T08:00:48Z","timestamp":1718956848000},"page":"472-476","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Evans syndrome: Disease awareness and clinical management in a nation\u2010wide <scp>ITP<\/scp>\u2010<scp>NET<\/scp> survey"],"prefix":"10.1111","volume":"113","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0857-8379","authenticated-orcid":false,"given":"Bruno","family":"Fattizzo","sequence":"first","affiliation":[{"name":"Hematology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"},{"name":"Department of Oncology and Hemato\u2010Oncology University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Carrai","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Ospedaliero\u2010Universitaria Careggi  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Crugnola","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Ospedaliero\u2010Universitaria di Parma  Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erminia","family":"Baldacci","sequence":"additional","affiliation":[{"name":"Hematology Unit Policlinico Umberto I, Sapienza University  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Bellini","sequence":"additional","affiliation":[{"name":"Hematology Unit ASST Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Costanza","family":"Bosi","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale Guglielmo da Saliceto  Piacenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Buzzatti","sequence":"additional","affiliation":[{"name":"Hematology Unit, Department of Biomedicine and Prevention University \u201cTor Vergata\u201d  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Domenica","family":"Caramazza","sequence":"additional","affiliation":[{"name":"Hematology Unit Hospital \u201cOspedale di Circolo e Fondazione Macchi\u201d  Varese Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Carli","sequence":"additional","affiliation":[{"name":"Hematology Unit Center for Hemorrhagic and Thrombotic Diseases (CMET), Ospedale San Bortolo  Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4355-3954","authenticated-orcid":false,"given":"Monica","family":"Carpenedo","sequence":"additional","affiliation":[{"name":"Hematology Unit ASST Fatebenefratelli Sacco  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Clissa","sequence":"additional","affiliation":[{"name":"Hematology Unit Integrated University Hospital of Verona, Policlinico G.B. Rossi  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Danesin","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale Ca' Foncello  Treviso Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"De Paolis","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale Vito Fazzi  Lecce Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juri Alessandro","family":"Giannotta","sequence":"additional","affiliation":[{"name":"Internal Medicine, Haemostasis and Thrombosis Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Innao","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale Garibaldi\u2010Nesima, ARNAS Garibaldi  Catania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7615-0572","authenticated-orcid":false,"given":"Monia","family":"Marchetti","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Ospedaliero\u2010Universitaria di Alessandria  Alessandria Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uros","family":"Markovic","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Ospedaliero\u2010Universitaria G. Rodolico  San Marco\u2014Catania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Morotti","sequence":"additional","affiliation":[{"name":"Internal Medicine Division\u2014Hematology Ospedale San Luigi Gonzaga, Orbassano  Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariasanta","family":"Napolitano","sequence":"additional","affiliation":[{"name":"Hematology Unit with Transplant Policlinico Universitario Paolo Giaccone  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Patriarca","sequence":"additional","affiliation":[{"name":"Unit of Transplantation and Biocompatibility: Hematopoietic Stem Cell Biology and Transplantation Ospedale Maggiore della Carit\u00e0  Novara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loredana","family":"Pettine","sequence":"additional","affiliation":[{"name":"Hematology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Poloni","sequence":"additional","affiliation":[{"name":"Hematology Clinic Universit\u00e0 Politecnica delle Marche\u2014AOU delle Marche  Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Rivolti","sequence":"additional","affiliation":[{"name":"Hematology Clinic, CORE Oncology and Hematology Center Arcispedale Santa Maria Nuova  Reggio Emilia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Rossi","sequence":"additional","affiliation":[{"name":"Department of Radiological and Hematological Sciences Policlinico Universitario Agostino Gemelli, Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa Maria","family":"Santeremo","sequence":"additional","affiliation":[{"name":"Complex Hematology Unit with Transplant Ospedale Monsignor Dimiccoli  Barletta Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7181-447X","authenticated-orcid":false,"given":"Cristina","family":"Santoro","sequence":"additional","affiliation":[{"name":"Hematology Unit Policlinico Umberto I, Sapienza University  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Elena","family":"Zannier","sequence":"additional","affiliation":[{"name":"Hematology Clinic Azienda Sanitaria Universitaria Friuli Centrale ASU FC  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Zaja","sequence":"additional","affiliation":[{"name":"DSM (University Clinical Department of Medical, Surgical and Health Sciences) University of Trieste  Trieste Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Cantoni","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Sanitaria Grande Ospedale Metropolitano Niguarda  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Palandri","sequence":"additional","affiliation":[{"name":"Hematology Clinic Policlinico Sant'Orsola  Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerio","family":"De Stefano","sequence":"additional","affiliation":[{"name":"Department of Radiological and Hematological Sciences Policlinico Universitario Agostino Gemelli, Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,6,21]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.1951.03810010058005"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9123851"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25574"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-11-887141"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0231284"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2020.271106"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-04-215368"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021005610"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2019000966"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2019.100648"},{"issue":"4","key":"e_1_2_9_12_1","article-title":"The European guidelines on diagnosis and management of neutropenia in adults and children: a consensus between the European Hematology Association and the EuNet\u2010INNOCHRON COST action","volume":"7","author":"Fioredda F","year":"2023","journal-title":"Hema"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022016818"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27144"},{"issue":"2","key":"e_1_2_9_15_1","first-page":"89","article-title":"Evans syndrome in the SARS\u2010CoV\u20102 era: \u201cspringing up like mushrooms","volume":"20","author":"Fattizzo B","year":"2022","journal-title":"Blood Transfus"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.14256","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T15:17:29Z","timestamp":1728487049000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.14256"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,21]]},"references-count":14,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2024,10]]}},"alternative-id":["10.1111\/ejh.14256"],"URL":"https:\/\/doi.org\/10.1111\/ejh.14256","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,21]]},"assertion":[{"value":"2024-04-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-04","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}