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      Stiffer Spleen Predicts Higher Bone Marrow Fibrosis and Higher JAK2 Allele Burden in Patients With Myeloproliferative Neoplasms

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          Abstract

          A total of 63 myeloproliferative neoplasms [MPN; 9 polycythemia vera (PV), 32 essential thrombocythemia (ET), and 22 myelofibrosis (MF)] underwent spleen stiffness (SS) measurement by vibration-controlled transient elastography equipped with a novel spleen-dedicated module. Higher SS values significantly correlated with grade 2-3 bone marrow (BM) fibrosis ( p=0.035), with hemoglobin level <10 g/dl ( p=0.014) and with white blood cells ≥10,000/μl ( p=0.008). Median SS was significantly higher in MF patients compared to ET and PV ( p=0.015). SS also correlated with higher JAK2 variant allele frequency ( p=0.02). This study identifies SS as a potential noninvasive tool that reflects BM fibrosis and the mutational burden in MPN.

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          Most cited references12

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          The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.

          The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues was last updated in 2008. Since then, there have been numerous advances in the identification of unique biomarkers associated with some myeloid neoplasms and acute leukemias, largely derived from gene expression analysis and next-generation sequencing that can significantly improve the diagnostic criteria as well as the prognostic relevance of entities currently included in the WHO classification and that also suggest new entities that should be added. Therefore, there is a clear need for a revision to the current classification. The revisions to the categories of myeloid neoplasms and acute leukemia will be published in a monograph in 2016 and reflect a consensus of opinion of hematopathologists, hematologists, oncologists, and geneticists. The 2016 edition represents a revision of the prior classification rather than an entirely new classification and attempts to incorporate new clinical, prognostic, morphologic, immunophenotypic, and genetic data that have emerged since the last edition. The major changes in the classification and their rationale are presented here.
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            Measurement of spleen stiffness to evaluate portal hypertension and the presence of esophageal varices in patients with HCV-related cirrhosis.

            The hepatic vein pressure gradient (HVPG) is the standard used to determine the degree of portal hypertension (PH) and an important prognostic factor for patients with cirrhosis; HVPG values correlate with the presence of esophageal varices (EV). However, HVPG can only be accurately determined at specialized centers; noninvasive methods are needed to predict HVPG values and the presence of EV. We compared the diagnostic performance of spleen stiffness (SS) measurement by transient elastography with that of liver stiffness (LS) and of other recently proposed noninvasive tests.
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              Spleen stiffness in patients with cirrhosis in predicting esophageal varices.

              Screening for esophageal varices (EV) is recommended in patients with cirrhosis. Noninvasive tests had shown varying sensitivity (Se) and specificity (Sp) for predicting EV. Splenomegaly is a common finding in liver cirrhosis because of portal and splenic congestion. These changes can be quantified by transient elastography; hence, the aim of this study was to investigate the utility of spleen stiffness (SS) in evaluating EV in comparison with other noninvasive tests.
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                Author and article information

                Contributors
                Journal
                Front Oncol
                Front Oncol
                Front. Oncol.
                Frontiers in Oncology
                Frontiers Media S.A.
                2234-943X
                26 October 2021
                2021
                : 11
                : 777730
                Affiliations
                [1] 1Division of Hematology, Department of Translational Medicine, Università del Piemonte Orientale and Azienda Ospedaliero-Universitaria Maggiore della Carità , Novara, Italy
                [2] 2Division of Internal Medicine, Department of Translational Medicine, Università del Piemonte Orientale and Azienda Ospedaliero-Universitaria Maggiore della Carità , Novara, Italy
                Author notes

                Edited by: Mohamed A. Yassin, Hamad Medical Corporation, Qatar

                Reviewed by: Alessandra Iurlo, IRCCS Ca ‘Granda Foundation Maggiore Policlinico Hospital, Italy; Giuseppe Alberto Palumbo, University of Catania, Italy

                *Correspondence: Cristina Rigamonti, cristina.rigamonti@ 123456uniupo.it

                This article was submitted to Hematologic Malignancies, a section of the journal Frontiers in Oncology

                †These authors have contributed equally to this work and share first authorship

                ‡These authors have contributed equally to this work and share senior authorship

                Article
                10.3389/fonc.2021.777730
                8576346
                34765563
                053dc3b2-a9fc-45e6-a8f4-08dc503a2a43
                Copyright © 2021 Moia, Cittone, Boggione, Manfredi, Favini, Awikeh, Pedrinelli, Mahmoud, Nicolosi, Bellan, Sainaghi, Pirisi, Gaidano, Patriarca and Rigamonti

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 15 September 2021
                : 08 October 2021
                Page count
                Figures: 1, Tables: 1, Equations: 0, References: 12, Pages: 5, Words: 2994
                Categories
                Oncology
                Brief Research Report

                Oncology & Radiotherapy
                myeloproliferative neoplasms,spleen stiffness,jak2 mutations,vibration-controlled transient elastography (vcte),ngs

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