{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T17:41:49Z","timestamp":1789062109697,"version":"build-2803163510"},"reference-count":33,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2015,10,12]],"date-time":"2015-10-12T00:00:00Z","timestamp":1444608000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Fondo de Investigaciones Sanitarias (FIS)"},{"name":"RTICC of the Instituto de Salud Carlos III"},{"name":"Ministry of Economy and Competitivity, Madrid","award":["PI11\/02399"],"award-info":[{"award-number":["PI11\/02399"]}]},{"name":"Ministry of Economy and Competitivity, Madrid","award":["RD12\/0036\/0048"],"award-info":[{"award-number":["RD12\/0036\/0048"]}]},{"name":"Ayudas a Proyectos de Investigaci\u00f3n en Salud de la Fundaci\u00f3n Mutua Madrile\u00f1a 2014"},{"name":"Ayuda de la Asociaci\u00f3n Espa\u00f1ola de Enfermos de Mastocitosis (AEDM 2014)"},{"name":"Fundacion Ramon Areces, Madrid","award":["CIVP16A1806"],"award-info":[{"award-number":["CIVP16A1806"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The diagnosis of \u2018rare diseases\u2019, such as mastocytosis, remains a challenge. Despite this, the precise benefits of referral of mastocytosis patients to highly specialized reference centres are poorly defined and whether patients should be managed at non\u2010specialized <jats:italic>versus<\/jats:italic> reference centres remains a matter of debate. To evaluate the quality and efficiency of diagnostic procedures performed at the reference centres for mastocytosis in Spain (<jats:styled-content style=\"fixed-case\">REMA<\/jats:styled-content>) <jats:italic>versus<\/jats:italic> other non\u2010reference centres, we retrospectively analysed a series of 122 patients, for the overall degree of agreement obtained for the World Health Organization (<jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content>) diagnostic and classification criteria betwen the referring and <jats:styled-content style=\"fixed-case\">REMA<\/jats:styled-content> centres. Our results showed that not all <jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content> diagnostic criteria were frequently investigated at the referring centres. Among the five <jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content> diagnostic criteria, the highest degree of agreement was obtained for serum tryptase levels [median 90% (95% confidence interval 84\u201396%)]; in turn, the overall agreement was significantly lower for the major histopathological criterion [80% (72\u201389%)], and the other three minor criteria: cytomorphology [68% (56\u201380%)] immunophenotyping of <jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content> mast cells [75% (62\u201387%)] and detection of the <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content> mutation [34% (8\u201360%)]. Referral of patients with diagnostic suspicion of mastocytosis to a multidisciplinary reference centre improves diagnostic efficiency and quality.<\/jats:p>","DOI":"10.1111\/bjh.13789","type":"journal-article","created":{"date-parts":[[2015,10,13]],"date-time":"2015-10-13T03:32:59Z","timestamp":1444707179000},"page":"56-63","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Diagnosis and classification of mastocytosis in non\u2010specialized <i>versus<\/i> reference centres: a Spanish Network on Mastocytosis (<scp>REMA<\/scp>) study on 122 patients"],"prefix":"10.1111","volume":"172","author":[{"given":"Laura","family":"S\u00e1nchez\u2010Mu\u00f1oz","sequence":"first","affiliation":[{"name":"Instituto de Estudios de Mastocitosis de Castilla La Mancha Hospital Virgen del Valle Toledo Spain"},{"name":"Spanish Network on Mastocytosis (REMA) Toledo and Salamanca 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Mastocytosis (REMA) Toledo and Salamanca Spain"},{"name":"Servicio General de Citometr\u00eda Centro de Investigaci\u00f3n del C\u00e1ncer (IBMCC\u2010CSIC\/USAL and IBSAL) and Departamento de Medicina Universidad de Salamanca Salamanca Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Mayado","sequence":"additional","affiliation":[{"name":"Spanish Network on Mastocytosis (REMA) Toledo and Salamanca Spain"},{"name":"Servicio General de Citometr\u00eda Centro de Investigaci\u00f3n del C\u00e1ncer (IBMCC\u2010CSIC\/USAL and IBSAL) and Departamento de Medicina Universidad de Salamanca Salamanca Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Mollejo","sequence":"additional","affiliation":[{"name":"Department of Pathology Hospital Virgen de la Salud Toledo Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Caldas","sequence":"additional","affiliation":[{"name":"Spanish 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