Special Issue "New Insights into Myeloproliferative Neoplasms"

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: 30 June 2020.

Special Issue Editor

Prof. Dr. Hans Hasselbalch
Website
Guest Editor
Department of Hematology, Zealand University Hospital, University of Copenhagen, Roskilde, Denmark
Interests: chronic myeloproliferative neoplasms (MPNs); gene expression profiling; epigenetics; SNPs; immune cell and molecular studies in MPNs; epidemiological studies; interferon-alpha2 (IFN-alpha2); statins; ruxolitinib in the treatment of MPNs

Special Issue Information

Dear Colleagues,

In recent years, new insights into the Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) have emerged, including the important role of chronic inflammation as the driving force for clonal evolution and disease progression and the impact of chronic inflammation upon symptom burden as well. The role of interferon-alfa2 (IFN) in the initial treatment of MPNs has been increasingly recognized, being fueled by the EU marketing authorization for RopegInterferon Alfa2b (BesremiR) as first line monotherapy in adults for the treatment of PV without symptomatic splenomegaly. The “wait and watch strategy” in low-risk patients is currently being challenged, since treatment with IFN may induce minimal residual disease (MRD) with low-burden JAK2V617F and normal bone marrow after about five years of IFN-treatment. Next generation sequencing is being used increasingly at the time of diagnosis for early prognostication and guidance for treatment. Most recent studies have unraveled MPNs to be far more prevalent than previously recognized, underscoring the unmet need of much earlier diagnosis of MPNs by molecular screening of patients at risk of having undiagnosed MPNs. This might also imply an earlier diagnosis of second cancers which MPN-patients are prone to develop, likely as a consequence of chronic inflammation, immunoderegulation and associated defective tumor immune surveillance.

This Special Issue will highlight several of the above issues, including mathematical modelling studies, substantiating the proof of concept for chronic inflammation as a trigger and driver of MPN development, and underscoring the importance of initiating IFN-treatment as early as possible. A new bright future for patients with MPNs is foreseen, in whom early intervention with stem cell targeted therapy (IFN) or IFN in combination with agents targeting the chronic inflammatory state (e.g., ruxolitinib and statins) may open the avenue for many more patients to follow the path towards MRD, and even cure being obtained by vaccination strategies.

Prof. Dr. Hans Hasselbalch
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All papers will be peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2000 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Philadelphia-negative myeloproliferative neoplasms (MPNs)
  • chronic inflammation
  • comorbidity burden
  • quality of life
  • early diagnosis and treatment
  • immune therapy
  • interferon-alpha2
  • vaccination
  • minimal residual disease
  • cure

Published Papers (2 papers)

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Research

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Open AccessArticle
Thrombotic Risk Detection in Patients with Polycythemia Vera: The Predictive Role of DNMT3A/TET2/ASXL1 Mutations
Cancers 2020, 12(4), 934; https://doi.org/10.3390/cancers12040934 - 10 Apr 2020
Abstract
The development of thrombotic events is common among patients with polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). We studied the influence of pathogenic mutations frequently associated with myeloid malignancies on thrombotic events using next-generation sequencing (NGS) in an initial cohort [...] Read more.
The development of thrombotic events is common among patients with polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). We studied the influence of pathogenic mutations frequently associated with myeloid malignancies on thrombotic events using next-generation sequencing (NGS) in an initial cohort of 68 patients with myeloproliferative neoplasms (MPN). As expected, the presence of mutations in DNMT3A, TET2, and ASXL1 (DTA genes) was positively associated with age for the whole cohort (p = 0.025, OR: 1.047, 95% CI: 1.006–1.090). Also, while not related with events in the whole cohort, DTA mutations were strongly associated with the development of vascular events in PV patients (p = 0.028). To confirm the possible association between the presence of DTA mutation and thrombotic events, we performed a case-control study on 55 age-matched patients with PV (including 12 PV patients from the initial cohort, 25 with event vs. 30 no event). In the age-matched case-control PV cohort, the presence of ≥1 DTA mutation significantly increased the risk of a thrombotic event (OR: 6.333, p = 0.0024). Specifically, mutations in TET2 were associated with thrombotic events in the PV case-control cohort (OR: 3.56, 95% CI: 1.15–11.83, p = 0.031). Our results suggest that pathogenic DTA mutations, and particularly TET2 mutations, may be an independent risk factor for thrombosis in patients with PV. However, the predictive value of TET2 and DTA mutations in ET and PMF was inconclusive and should be determined in a larger cohort. Full article
(This article belongs to the Special Issue New Insights into Myeloproliferative Neoplasms)
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Review

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Open AccessReview
Ocular Manifestations in Patients with Philadelphia-Negative Myeloproliferative Neoplasms
Cancers 2020, 12(3), 573; https://doi.org/10.3390/cancers12030573 - 02 Mar 2020
Abstract
The major complications of Philadelphia-negative (Ph-Negative) myeloproliferative neoplasms (MPNs) are thrombosis, haemorrhage and leukemic transformation. As systemic and haematological diseases, MPNs have the potential to affect many tissues and organs. Some complications lead to the diagnosis of MPNs, but other signs and symptoms [...] Read more.
The major complications of Philadelphia-negative (Ph-Negative) myeloproliferative neoplasms (MPNs) are thrombosis, haemorrhage and leukemic transformation. As systemic and haematological diseases, MPNs have the potential to affect many tissues and organs. Some complications lead to the diagnosis of MPNs, but other signs and symptoms are often misdiagnosed or neglected as a sign of MPN disease. Therefore, we reviewed the current literature to investigate and delineate the clinical manifestations seen in the eyes of Ph-negative MPN patients. We found that ocular manifestations are common among patients with MPNs. The most frequently described manifestations are due to the consequences of haematological abnormalities causing microvascular disturbances and hyperviscosity. More serious and vision-threatening complications as thrombotic events in the eyes have been repeatedly reported as well. These ocular symptoms may precede more serious extraocular complications. Accordingly, combined ophthalmological and haematological management have the potential to discover these diseases earlier and prevent morbidity and mortality in these patients. Furthermore, routine ophthalmological screening of all newly diagnosed MPN patients may be a preventive approach for early diagnosis and timely treatment of the ocular manifestations. Full article
(This article belongs to the Special Issue New Insights into Myeloproliferative Neoplasms)
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