Is there a gender effect in polycythemia vera?

Palandri, Francesca; Mora, Barbara; Gangat, Naseema; Catani, Lucia

doi:10.1007/s00277-020-04287-w

Cited by 13 publications

(10 citation statements)

References 121 publications

(230 reference statements)

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“…Previous gene expression analysis by oligonucleotide microarray has revealed that men with PV have more differentially expressed genes than women, suggesting that gender may be a confounding factor in expression profiling 31 . Also, PV pathogenesis, phenotype, and prognosis are all considered to be gender-biased 32 . Unfortunately, the gender of our patient cohort was predominantly men, which reflects the higher prevalence of PV in males but may confound protein expression studies.…”

Section: Discussionmentioning

confidence: 99%

MTAP-related increased erythroblast proliferation as a mechanism of polycythaemia vera

Tipgomut

Khuhapinant

Wilson

et al. 2021

Sci Rep

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Polycythaemia vera (PV) is a haematological disorder caused by an overproduction of erythroid cells. To date, the molecular mechanisms involved in the disease pathogenesis are still ambiguous. This study aims to identify aberrantly expressed proteins in erythroblasts of PV patients by utilizing mass spectrometry-based proteomic analysis. Haematopoietic stem cells (HSCs) were isolated from newly-diagnosed PV patients, PV patients who have received cytoreductive therapy, and healthy subjects. In vitro erythroblast expansion confirmed that the isolated HSCs recapitulated the disease phenotype as the number of erythroblasts from newly-diagnosed PV patients was significantly higher than those from the other groups. Proteomic comparison revealed 17 proteins that were differentially expressed in the erythroblasts from the newly-diagnosed PV patients compared to those from healthy subjects, but which were restored to normal levels in the patients who had received cytoreductive therapy. One of these proteins was S-methyl-5′-thioadenosine phosphorylase (MTAP), which had reduced expression in PV patients’ erythroblasts. Furthermore, MTAP knockdown in normal erythroblasts was shown to enhance their proliferative capacity. Together, this study identifies differentially expressed proteins in erythroblasts of healthy subjects and those of PV patients, indicating that an alteration of protein expression in erythroblasts may be crucial to the pathology of PV.

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Section: Discussionmentioning

confidence: 99%

MTAP-related increased erythroblast proliferation as a mechanism of polycythaemia vera

Tipgomut

Khuhapinant

Wilson

et al. 2021

Sci Rep

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“…No association was found between BP incidence and HU or busulfan use [11]. Impact of gender on BP transformation is not clear [34,38].…”

Section: Predictive Factors Of Survival and Blast Phase Evolutionmentioning

confidence: 93%

“…Pruritus was identified as being prognostically favorable [11]. If the IWG-MRT model does not include gender among variables, another retrospective study showed that females are at lower risk of death, leaving this topic open to debate [34].…”

Section: Predictive Factors Of Survival and Blast Phase Evolutionmentioning

confidence: 99%

Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms

Mora

Passamonti

2022

Curr Hematol Malig Rep

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Purpose of Review Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic (pre-), and overt-primary myelofibrosis (primary MF, PMF). PV and ET could evolve into secondary MF (SMF), whose early diagnosis relies on monitoring signs of possible progression. All MPNs have a risk of blast phase (BP), that is associated with a very dismal outcome. Overall survival (OS) is different among MPNs, and disease-specific prognostic scores should be applied for a correct clinical management. In this review, an overview of current prognostic scores in MPNs will be provided. Recent Findings The biological complexity of MPNs and its role on the trajectory of disease outcome have led to the design of integrated prognostic models that are nowadays of common use in PMF patients. As for PV and ET, splicing gene mutations could have a detrimental role, but with the limit of the not routinary recommended application of extensive molecular analysis in these diseases. SMF is recognized as a distinct entity compared to PMF, and OS estimates should be calculated by the MYSEC-PM (Myelofibrosis SECondary-prognostic model). Both in PMF and SMF, decisions as selection of patients potentially candidates to allogenic stem cell transplant or that could benefit from an early shift from standard treatment are based not only on conventional prognostic scores, but also on multivariable algorithms. Summary The expanding landscape of risk prediction for OS, evolution to BP, and SMF progression from PV/ET informs personalized approach to the management of patients affected by MPNs.

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“…It has been shown that excess mortality in MPN patients is mainly due to death from hematologic malignancies or Figure nfections, and in young patients also from cerebrovascular and cardiovascular diseases [1][2][3][4][5][6]. Sex influences MPN presentation, symptom burden and natural history [7,8]. "Driver" mutations in three genes (Janus kinase (JAK)2, calreticulin (CALR) and myeloproliferative leukemia virus oncogene (MPL)) that activate the JAK2 signaling pathway have been detected in MPN.…”

Section: Introduction 1myeloproliferative Neoplasmsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Power of Extracellular Vesicles in Myeloproliferative Neoplasms: “Crafting” a Microenvironment That Matters

Catani

Cavo

Palandri³

2021

Cells

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Myeloproliferative Neoplasms (MPN) are acquired clonal disorders of the hematopoietic stem cells and include Essential Thrombocythemia, Polycythemia Vera and Myelofibrosis. MPN are characterized by mutations in three driver genes (JAK2, CALR and MPL) and by a state of chronic inflammation. Notably, MPN patients experience increased risk of thrombosis, disease progression, second neoplasia and evolution to acute leukemia. Extracellular vesicles (EVs) are a heterogeneous population of microparticles with a role in cell-cell communication. The EV-mediated cross-talk occurs via the trafficking of bioactive molecules such as nucleic acids, proteins, metabolites and lipids. Growing interest is focused on EVs and their potential impact on the regulation of blood cancers. Overall, EVs have been suggested to orchestrate the complex interplay between tumor cells and the microenvironment with a pivotal role in “education” and “crafting” of the microenvironment by regulating angiogenesis, coagulation, immune escape and drug resistance of tumors. This review is focused on the role of EVs in MPN. Specifically, we will provide an overview of recent findings on the involvement of EVs in MPN pathogenesis and discuss opportunities for their potential application as diagnostic and prognostic biomarkers.

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Is there a gender effect in polycythemia vera?

Cited by 13 publications

References 121 publications

MTAP-related increased erythroblast proliferation as a mechanism of polycythaemia vera

MTAP-related increased erythroblast proliferation as a mechanism of polycythaemia vera

Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms

The Power of Extracellular Vesicles in Myeloproliferative Neoplasms: “Crafting” a Microenvironment That Matters

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