New Markers of Disease Progression in Myelofibrosis

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm due to the clonal proliferation of a hematopoietic stem cell. The vast majority of patients harbor a somatic gain of function mutation either of JAK2 or MPL or CALR genes in their he...

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Autores principales:	Rita Campanelli, Margherita Massa, Vittorio Rosti, Giovanni Barosi
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	chronic myeloproliferative neoplasms primary myelofibrosis inflammation prognosis Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/41225f8fcfb34c65adcfa620ede973bc
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spelling	oai:doaj.org-article:41225f8fcfb34c65adcfa620ede973bc2021-11-11T15:28:19ZNew Markers of Disease Progression in Myelofibrosis10.3390/cancers132153242072-6694https://doaj.org/article/41225f8fcfb34c65adcfa620ede973bc2021-10-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/21/5324https://doaj.org/toc/2072-6694Primary myelofibrosis (PMF) is a myeloproliferative neoplasm due to the clonal proliferation of a hematopoietic stem cell. The vast majority of patients harbor a somatic gain of function mutation either of <i>JAK2</i> or <i>MPL</i> or <i>CALR</i> genes in their hematopoietic cells, resulting in the activation of the JAK/STAT pathway. Patients display variable clinical and laboratoristic features, including anemia, thrombocytopenia, splenomegaly, thrombotic complications, systemic symptoms, and curtailed survival due to infections, thrombo-hemorrhagic events, or progression to leukemic transformation. New drugs have been developed in the last decade for the treatment of PMF-associated symptoms; however, the only curative option is currently represented by allogeneic hematopoietic cell transplantation, which can only be offered to a small percentage of patients. Disease prognosis is based at diagnosis on the classical International Prognostic Scoring System (IPSS) and Dynamic-IPSS (during disease course), which comprehend clinical parameters; recently, new prognostic scoring systems, including genetic and molecular parameters, have been proposed as meaningful tools for a better patient stratification. Moreover, new biological markers predicting clinical evolution and patient survival have been associated with the disease. This review summarizes basic concepts of PMF pathogenesis, clinics, and therapy, focusing on classical prognostic scoring systems and new biological markers of the disease.Rita CampanelliMargherita MassaVittorio RostiGiovanni BarosiMDPI AGarticlechronic myeloproliferative neoplasmsprimary myelofibrosisinflammationprognosisNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5324, p 5324 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	chronic myeloproliferative neoplasms primary myelofibrosis inflammation prognosis Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	chronic myeloproliferative neoplasms primary myelofibrosis inflammation prognosis Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Rita Campanelli Margherita Massa Vittorio Rosti Giovanni Barosi New Markers of Disease Progression in Myelofibrosis
description	Primary myelofibrosis (PMF) is a myeloproliferative neoplasm due to the clonal proliferation of a hematopoietic stem cell. The vast majority of patients harbor a somatic gain of function mutation either of <i>JAK2</i> or <i>MPL</i> or <i>CALR</i> genes in their hematopoietic cells, resulting in the activation of the JAK/STAT pathway. Patients display variable clinical and laboratoristic features, including anemia, thrombocytopenia, splenomegaly, thrombotic complications, systemic symptoms, and curtailed survival due to infections, thrombo-hemorrhagic events, or progression to leukemic transformation. New drugs have been developed in the last decade for the treatment of PMF-associated symptoms; however, the only curative option is currently represented by allogeneic hematopoietic cell transplantation, which can only be offered to a small percentage of patients. Disease prognosis is based at diagnosis on the classical International Prognostic Scoring System (IPSS) and Dynamic-IPSS (during disease course), which comprehend clinical parameters; recently, new prognostic scoring systems, including genetic and molecular parameters, have been proposed as meaningful tools for a better patient stratification. Moreover, new biological markers predicting clinical evolution and patient survival have been associated with the disease. This review summarizes basic concepts of PMF pathogenesis, clinics, and therapy, focusing on classical prognostic scoring systems and new biological markers of the disease.
format	article
author	Rita Campanelli Margherita Massa Vittorio Rosti Giovanni Barosi
author_facet	Rita Campanelli Margherita Massa Vittorio Rosti Giovanni Barosi
author_sort	Rita Campanelli
title	New Markers of Disease Progression in Myelofibrosis
title_short	New Markers of Disease Progression in Myelofibrosis
title_full	New Markers of Disease Progression in Myelofibrosis
title_fullStr	New Markers of Disease Progression in Myelofibrosis
title_full_unstemmed	New Markers of Disease Progression in Myelofibrosis
title_sort	new markers of disease progression in myelofibrosis
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/41225f8fcfb34c65adcfa620ede973bc
work_keys_str_mv	AT ritacampanelli newmarkersofdiseaseprogressioninmyelofibrosis AT margheritamassa newmarkersofdiseaseprogressioninmyelofibrosis AT vittoriorosti newmarkersofdiseaseprogressioninmyelofibrosis AT giovannibarosi newmarkersofdiseaseprogressioninmyelofibrosis
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New Markers of Disease Progression in Myelofibrosis

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