Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants

Progression in Ph-chromosome-negative myeloproliferative neoplasms (MPN) develops with variable incidence and time sequence in essential thrombocythemia, polycythemia vera, and primary myelofibrosis. These diseases show different clinic-pathologic features and outcomes despite sharing deregulated JA...

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Autores principales:	Elena Sabattini, Marco Pizzi, Claudio Agostinelli, Clara Bertuzzi, Carlo Alberto Sagramoso Sacchetti, Francesca Palandri, Umberto Gianelli
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	myeloproliferative neoplasms WHO classification progression fibrosis leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/a89b9419b11b44bb948354de6c0651f4
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spelling	oai:doaj.org-article:a89b9419b11b44bb948354de6c0651f42021-11-11T15:33:58ZProgression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants10.3390/cancers132155312072-6694https://doaj.org/article/a89b9419b11b44bb948354de6c0651f42021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/21/5531https://doaj.org/toc/2072-6694Progression in Ph-chromosome-negative myeloproliferative neoplasms (MPN) develops with variable incidence and time sequence in essential thrombocythemia, polycythemia vera, and primary myelofibrosis. These diseases show different clinic-pathologic features and outcomes despite sharing deregulated JAK/STAT signaling due to mutations in either the Janus kinase 2 or myeloproliferative leukemia or <i>CALR</i>eticulin genes, which are the primary drivers of the diseases, as well as defined diagnostic criteria and biomarkers in most cases. Progression is defined by the development or worsening of marrow fibrosis or the progressive increase in the marrow blast percentage. Progression is often related to additional genetic aberrations, although some can already be detected during the chronic phase. Detailed scoring systems for clinical usage that are mostly applied in patients with primary myelofibrosis have been defined, and the most recent ones include cytogenetic and molecular parameters with prognostic significance. Additional different clinic-pathologic changes have been reported that may occur during the course of the disease and that are, at present, classified as WHO-defined types of progression, although they likely represent such an event. The present review is meant to provide an updated overview on progression in Ph-chromosome-negative MPN, with a major focus on the pathologic side.Elena SabattiniMarco PizziClaudio AgostinelliClara BertuzziCarlo Alberto Sagramoso SacchettiFrancesca PalandriUmberto GianelliMDPI AGarticlemyeloproliferative neoplasmsWHO classificationprogressionfibrosisleukemiaNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5531, p 5531 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	myeloproliferative neoplasms WHO classification progression fibrosis leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	myeloproliferative neoplasms WHO classification progression fibrosis leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Elena Sabattini Marco Pizzi Claudio Agostinelli Clara Bertuzzi Carlo Alberto Sagramoso Sacchetti Francesca Palandri Umberto Gianelli Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
description	Progression in Ph-chromosome-negative myeloproliferative neoplasms (MPN) develops with variable incidence and time sequence in essential thrombocythemia, polycythemia vera, and primary myelofibrosis. These diseases show different clinic-pathologic features and outcomes despite sharing deregulated JAK/STAT signaling due to mutations in either the Janus kinase 2 or myeloproliferative leukemia or <i>CALR</i>eticulin genes, which are the primary drivers of the diseases, as well as defined diagnostic criteria and biomarkers in most cases. Progression is defined by the development or worsening of marrow fibrosis or the progressive increase in the marrow blast percentage. Progression is often related to additional genetic aberrations, although some can already be detected during the chronic phase. Detailed scoring systems for clinical usage that are mostly applied in patients with primary myelofibrosis have been defined, and the most recent ones include cytogenetic and molecular parameters with prognostic significance. Additional different clinic-pathologic changes have been reported that may occur during the course of the disease and that are, at present, classified as WHO-defined types of progression, although they likely represent such an event. The present review is meant to provide an updated overview on progression in Ph-chromosome-negative MPN, with a major focus on the pathologic side.
format	article
author	Elena Sabattini Marco Pizzi Claudio Agostinelli Clara Bertuzzi Carlo Alberto Sagramoso Sacchetti Francesca Palandri Umberto Gianelli
author_facet	Elena Sabattini Marco Pizzi Claudio Agostinelli Clara Bertuzzi Carlo Alberto Sagramoso Sacchetti Francesca Palandri Umberto Gianelli
author_sort	Elena Sabattini
title	Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
title_short	Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
title_full	Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
title_fullStr	Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
title_full_unstemmed	Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants
title_sort	progression in ph-chromosome-negative myeloproliferative neoplasms: an overview on pathologic issues and molecular determinants
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/a89b9419b11b44bb948354de6c0651f4
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Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants

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