{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T21:30:51Z","timestamp":1775079051651,"version":"3.50.1"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2023,3,4]],"date-time":"2023-03-04T00:00:00Z","timestamp":1677888000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,3,4]],"date-time":"2023-03-04T00:00:00Z","timestamp":1677888000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Leukemia"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In this <jats:italic>Perspective<\/jats:italic>, we discuss criteria for defining a new disease entity or variant of a recognized disease or disorder. We do so in the context of the current topography of the BCR::ABL-negative myeloproliferative neoplasms (MPNs) where two new variants are reported: <jats:italic>clonal megakaryocyte dysplasia with normal blood values<\/jats:italic> (CMD-NBV) and <jats:italic>clonal megakaryocyte dysplasia with isolated thrombocytosis<\/jats:italic> (CMD-IT). The cardinal feature of these variants is bone marrow megakaryocyte hyperplasia and atypia corresponding the WHO histological criteria for primary myelofibrosis (<jats:italic>myelofibrosis-type megakaryocyte dysplasia-MTMD<\/jats:italic>). Persons with these new variants have a different disease course and features from others in the MPN domain. In a broader context we suggest myelofibrosis-type megakaryocyte dysplasia defines a spectrum of related MPN variants including CMD-NBV, CMD-IT, pre-fibrotic myelofibrosis and overt myelofibrosis, which differ from polycythemia vera and essential thrombocythemia. Our proposal needs external validation and we stress the need for a consensus definition of the megakaryocyte dysplasia which is the hallmark of these disorders.<\/jats:p>","DOI":"10.1038\/s41375-023-01861-9","type":"journal-article","created":{"date-parts":[[2023,3,4]],"date-time":"2023-03-04T08:02:44Z","timestamp":1677916964000},"page":"725-727","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Myelofibrosis-type megakaryocyte dysplasia (MTMD) as a distinct category of BCR::ABL-negative myeloproliferative neoplasms. Challenges and perspectives"],"prefix":"10.1038","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8357-7192","authenticated-orcid":false,"given":"Giovanni","family":"Barosi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vittorio","family":"Rosti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9156-1676","authenticated-orcid":false,"given":"Robert Peter","family":"Gale","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,3,4]]},"reference":[{"key":"1861_CR1","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1159\/000517207","volume":"145","author":"G Barosi","year":"2022","unstructured":"Barosi G, Rosti V, Massa M, Campanelli R, Villani L, Catarsi P, et al. Clonal Megakaryocyte Dysplasia with Normal Blood Values Is a Distinct Myeloproliferative Neoplasm. Acta Haematol. 2022;145:30\u20137.","journal-title":"Acta Haematol."},{"key":"1861_CR2","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1159\/000527284","volume":"146","author":"G Barosi","year":"2023","unstructured":"Barosi G, Campanelli R, Massa M, Catarsi P, Carolei A, Abb\u00e0 C, et al. Clonal Megakaryocyte Dysplasia with Isolated Thrombocytosis Is a Distinct Myeloproliferative Neoplasm Phenotype. Acta Haematol. 2023;146:14\u201325.","journal-title":"Acta Haematol."},{"key":"1861_CR3","doi-asserted-by":"publisher","first-page":"2310","DOI":"10.1002\/1097-0142(19911115)68:10<2310::AID-CNCR2820681034>3.0.CO;2-2","volume":"68","author":"G Barosi","year":"1991","unstructured":"Barosi G, Buratti A, Costa A, Liberato LN, Balduini C, Cazzola M, et al. An atypical myeloproliferative disorder with high thrombotic risk and slow disease progression. 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