{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T04:38:41Z","timestamp":1792643921606,"version":"4.5.0"},"reference-count":27,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,6,9]],"date-time":"2021-06-09T00:00:00Z","timestamp":1623196800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>A retrospective population\u2010based study to determine the incidence and prevalence of patients with the rare blood disease paroxysmal nocturnal haemoglobinuria (PNH).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>All patients were identified by flow cytometric detection of blood cells deficient in glycosylphosphatidylinositol (GPI) linked proteins at a single diagnostic reference laboratory that serves the Yorkshire based, Haematological Malignancy Research Network (HMRN) with a population of 3.8\u00a0million.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hundred and ninety\u2010seven patients with detectable PNH clones at a level of &gt;0.01% in at least two lineages of cells (neutrophils, monocytes and\/or red cells) were identified over a 15\u2010year period (2004\u20102018). Of these, 88% had aplastic anaemia (AA), 8% classical PNH and 3% myelodysplastic syndrome. The overall incidence rate was estimated at 0.35 cases per 100\u00a0000 people per year. This equates to 220 cases newly diagnosed in the United Kingdom each year. The overall prevalence rate was 3.81 per 100\u00a0000, this equates to an estimated 2400 prevalent cases in the UK. The overall and relative 5\u2010year survival rates were 72% and 82.7%, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study showed that classical haemolytic PNH is a rare disease and represents only a small proportion overall of patients with detectable PNH cells, the majority of which have aplastic anaemia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13640","type":"journal-article","created":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T11:51:09Z","timestamp":1622548269000},"page":"211-218","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":64,"title":["The incidence and prevalence of patients with paroxysmal nocturnal haemoglobinuria and aplastic anaemia PNH syndrome: A retrospective analysis of the UK\u2019s population\u2010based haematological malignancy research network 2004\u20102018"],"prefix":"10.1111","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8421-5353","authenticated-orcid":false,"given":"Stephen J.","family":"Richards","sequence":"first","affiliation":[{"name":"Division of Haematology and Immunology Leeds Institute of Medical Research at St James's University of Leeds Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Painter","sequence":"additional","affiliation":[{"name":"Epidemiology and Cancer Statistics Group Department of Health Sciences University of York York UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita J.","family":"Dickinson","sequence":"additional","affiliation":[{"name":"Haematological Malignancy Diagnostic Service Leeds Teaching Hospitals NHS Trust St. James's University Hospital Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morag","family":"Griffin","sequence":"additional","affiliation":[{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talha","family":"Munir","sequence":"additional","affiliation":[{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise","family":"Arnold","sequence":"additional","affiliation":[{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Payne","sequence":"additional","affiliation":[{"name":"Haematological Malignancy Diagnostic Service Leeds Teaching Hospitals NHS Trust St. James's University Hospital Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Pike","sequence":"additional","affiliation":[{"name":"Division of Haematology and Immunology Leeds Institute of Medical Research at St James's University of Leeds Leeds UK"},{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Muus","sequence":"additional","affiliation":[{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"Hill","sequence":"additional","affiliation":[{"name":"Department of Haematology Leeds Teaching Hospitals NHS Trust Leeds UK"},{"name":"Alexion Pharmaceuticals Inc. 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