The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases

Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic stem cell disorders, characterized by increased proliferation of one or more myeloid lineages in the bone marrow. The classification and diagnostic criteria of MPNs have undergone relevant changes over the years, r...

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Autores principales:	Marco Pizzi, Giorgio Alberto Croci, Marco Ruggeri, Silvia Tabano, Angelo Paolo Dei Tos, Elena Sabattini, Umberto Gianelli
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	Myeloproliferative neoplasms WHO Classification MPN-U myeloid disorders Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/545e874a0b13499898b3a4d0e53729eb
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spelling	oai:doaj.org-article:545e874a0b13499898b3a4d0e53729eb2021-11-25T17:02:26ZThe Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases10.3390/cancers132256662072-6694https://doaj.org/article/545e874a0b13499898b3a4d0e53729eb2021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/22/5666https://doaj.org/toc/2072-6694Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic stem cell disorders, characterized by increased proliferation of one or more myeloid lineages in the bone marrow. The classification and diagnostic criteria of MPNs have undergone relevant changes over the years, reflecting the increased awareness on these conditions and a better understanding of their biological and clinical-pathological features. The current World Health Organization (WHO) Classification acknowledges four main sub-groups of MPNs: (i) Chronic Myeloid Leukemia; (ii) classical Philadelphia-negative MPNs (Polycythemia Vera; Essential Thrombocythemia; Primary Myelofibrosis); (iii) non-classical Philadelphia-negative MPNs (Chronic Neutrophilic Leukemia; Chronic Eosinophilic Leukemia); and (iv) MPNs, unclassifiable (MPN-U). The latter are currently defined as MPNs with clinical-pathological findings not fulfilling the diagnostic criteria for any other entity. The MPN-U spectrum traditionally encompasses early phase MPNs, terminal (i.e., advanced fibrotic) MPNs, and cases associated with inflammatory or neoplastic disorders that obscure the clinical-histological picture. Several lines of evidence and clinical practice suggest the existence of additional myeloid neoplasms that may expand the spectrum of MPN-U. To gain insight into such disorders, this review addresses the history of MPN classification, the evolution of their diagnostic criteria and the complex clinical-pathological and biological features of MPN-U.Marco PizziGiorgio Alberto CrociMarco RuggeriSilvia TabanoAngelo Paolo Dei TosElena SabattiniUmberto GianelliMDPI AGarticleMyeloproliferative neoplasmsWHO ClassificationMPN-Umyeloid disordersNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5666, p 5666 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Myeloproliferative neoplasms WHO Classification MPN-U myeloid disorders Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	Myeloproliferative neoplasms WHO Classification MPN-U myeloid disorders Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Marco Pizzi Giorgio Alberto Croci Marco Ruggeri Silvia Tabano Angelo Paolo Dei Tos Elena Sabattini Umberto Gianelli The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
description	Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic stem cell disorders, characterized by increased proliferation of one or more myeloid lineages in the bone marrow. The classification and diagnostic criteria of MPNs have undergone relevant changes over the years, reflecting the increased awareness on these conditions and a better understanding of their biological and clinical-pathological features. The current World Health Organization (WHO) Classification acknowledges four main sub-groups of MPNs: (i) Chronic Myeloid Leukemia; (ii) classical Philadelphia-negative MPNs (Polycythemia Vera; Essential Thrombocythemia; Primary Myelofibrosis); (iii) non-classical Philadelphia-negative MPNs (Chronic Neutrophilic Leukemia; Chronic Eosinophilic Leukemia); and (iv) MPNs, unclassifiable (MPN-U). The latter are currently defined as MPNs with clinical-pathological findings not fulfilling the diagnostic criteria for any other entity. The MPN-U spectrum traditionally encompasses early phase MPNs, terminal (i.e., advanced fibrotic) MPNs, and cases associated with inflammatory or neoplastic disorders that obscure the clinical-histological picture. Several lines of evidence and clinical practice suggest the existence of additional myeloid neoplasms that may expand the spectrum of MPN-U. To gain insight into such disorders, this review addresses the history of MPN classification, the evolution of their diagnostic criteria and the complex clinical-pathological and biological features of MPN-U.
format	article
author	Marco Pizzi Giorgio Alberto Croci Marco Ruggeri Silvia Tabano Angelo Paolo Dei Tos Elena Sabattini Umberto Gianelli
author_facet	Marco Pizzi Giorgio Alberto Croci Marco Ruggeri Silvia Tabano Angelo Paolo Dei Tos Elena Sabattini Umberto Gianelli
author_sort	Marco Pizzi
title	The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
title_short	The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
title_full	The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
title_fullStr	The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
title_full_unstemmed	The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases
title_sort	classification of myeloproliferative neoplasms: rationale, historical background and future perspectives with focus on unclassifiable cases
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/545e874a0b13499898b3a4d0e53729eb
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The Classification of Myeloproliferative Neoplasms: Rationale, Historical Background and Future Perspectives with Focus on Unclassifiable Cases

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