{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T05:17:15Z","timestamp":1788239835559,"version":"build-2803163510"},"reference-count":70,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T00:00:00Z","timestamp":1788134400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T00:00:00Z","timestamp":1788134400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by a defect of glycosylphosphatidyl\u2010anchored proteins, leading to an uncontrolled complement\u2010mediated hemolysis. The advent of complement inhibitors in clinical practice radically changed patients' outcomes and survival. Despite this significant progress, numerous unmet needs persist in clinical practice. A group of Italian experts explored the main challenges currently associated with PNH diagnosis and monitoring and reached a consensus on screening, diagnosis, initial work\u2010up, and monitoring. The following core questions were examined: (i) Which patients need to be tested? (ii) Which test to use, and how do we define positivity? (iii) Which workup is requested for patients with a newly detected PNH clone? (iv) Which monitoring in untreated patients? (v) Which monitoring in C\u2010terminal\u2010inh and C\u2010proximal inh? Each specific clinical question was framed according to the GRADE format. Overall, flow\u2010cytometry for PNH detection was recommended in patients presenting with two or more suggestive signs or symptoms and\/or with hematologic conditions known to be associated with PNH clones, including DAT\u2010negative hemolysis, idiopathic venous thrombosis (particularly at atypical sites), and primary bone marrow dysfunction (such as aplastic anemia or myelodysplastic syndrome). A high\u2010sensitivity flow cytometry test in neutrophils and erythrocytes should be preferred (lower limit of quantification 0.01%). Baseline assessment of disease activity (i.e., LDH &gt;\u20091.5\u00d7ULN and PNH signs\/symptoms), thrombotic risk, bone marrow (BM) features and imaging for organ dysfunction are suggested. Monitoring comprises blood counts and hemolytic markers with frequency depending on anemia severity and symptoms, as well as annual PNH clone retesting, BM reevaluation in case of cytopenias, and constant education about infectious risk and breakthrough hemolysis in patients on treatment.<\/jats:p>","DOI":"10.1111\/ejh.70294","type":"journal-article","created":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T04:30:51Z","timestamp":1788237051000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Expert Consensus on the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria (\n                    <scp>PNH<\/scp>\n                    ): An Algorithmic Approach in an Era of New Treatments"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0857-8379","authenticated-orcid":false,"given":"Bruno","family":"Fattizzo","sequence":"first","affiliation":[{"name":"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"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7615-0572","authenticated-orcid":false,"given":"Monia","family":"Marchetti","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliera Nazionale SS Antonio e Biagio e Cesare Arrigo  Alessandria Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Cannizzo","sequence":"additional","affiliation":[{"name":"U.O.C. Servizio di Immunoematologia e Medicina Trasfusionale Azienda Sanitaria Provinciale di Ragusa  Ragusa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Gatti","sequence":"additional","affiliation":[{"name":"Hematology Laboratory and Transfusion Center ASST Ovest Milanese  Legnano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6475-5547","authenticated-orcid":false,"given":"Massimo","family":"Geuna","sequence":"additional","affiliation":[{"name":"Independent Researcher  Torino Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1216-5270","authenticated-orcid":false,"given":"Anna Paola","family":"Iori","sequence":"additional","affiliation":[{"name":"Department of Hematology, Oncology, and Dermatology AOU Policlinico Umberto I  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maddalena","family":"Raia","sequence":"additional","affiliation":[{"name":"CEINGE Biotecnologie Avanzate Franco Salvatore S.C. A r.l  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2426-3465","authenticated-orcid":false,"given":"Simona","family":"Sica","sequence":"additional","affiliation":[{"name":"Department of Laboratory and Hematological Sciences Fondazione Policlinico Universitario A. Gemelli IRCCS  Rome Italy"},{"name":"Department of Radiological and Hematological Sciences Universit\u00e0 Cattolica del Sacro Cuore, Sezione di Ematologia  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4320-9253","authenticated-orcid":false,"given":"Francesco","family":"Buccisano","sequence":"additional","affiliation":[{"name":"Hematology, Department of Biomedicine and Prevention University of Rome \u201cTor Vergata\u201d  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,31]]},"reference":[{"issue":"9","key":"e_1_2_10_2_1","doi-asserted-by":"crossref","DOI":"10.3390\/medicina59091612","article-title":"Paroxysmal Nocturnal Hemoglobinuria: Biology and Treatment","volume":"59","author":"Bravo\u2010Perez C.","year":"2023","journal-title":"Medicina"},{"issue":"1","key":"e_1_2_10_3_1","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1172\/JCI114172","article-title":"Isolation and Characterization of a Membrane Protein From Normal Human Erythrocytes That Inhibits Reactive Lysis of the Erythrocytes of Paroxysmal Nocturnal Hemoglobinuria","volume":"84","author":"Holguin M. 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