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Cancers
Special Issues
Therapeutic Approaches for Myeloproliferative Neoplasms
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Therapeutic Approaches for Myeloproliferative Neoplasms

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: 30 April 2024 | Viewed by 1809

Special Issue Editors

Dr. Massimo Breccia

E-Mail Website
Guest Editor
Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
Interests: chronic myeloid leukemia; Ph-negative myeloproliferative disorders; MDS; AML; APL
Dr. Francesca Palandri

E-Mail Website
Guest Editor
Istituto di Ematologia “Seràgnoli”, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Interests: Ph-negative myeloproliferative disorders; immune thrombocytopenia

Special Issue Information

Dear Colleagues,

Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell myeloid neoplasms that are characterized by the proliferation and preserved differentiation of myeloid cell lineages. Excluding BCR-ABL1-positive chronic myeloid leukemia (CML), essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF) are the most frequent MPNs.

Constitutive activation of the Janus kinase 2 (JAK2)/signal transducer and activator of transcription (STAT) signaling pathway due to acquired somatic mutations in the JAK2, calreticulin or MPL genes may drive the course of MPNs. These mutations seem to confer the advantage of survival and proliferation to myeloid hematopoietic cells independently of stimulatory signals, leading to clonal expansion of myeloid progenitors and mature cells.

Traditional treatment approaches for PV and ET focus on the prevention of thrombotic events with aspirin along with antiproliferative agents. Treatment strategies for MF are driven by clinical presentations such as anemia and/or splenomegaly. In the last decade, improvement in the therapeutic approaches for MPN have been developed, in particular in PV and MF, with different new drugs on the horizon that will completely change the therapeutic algorithms for these diseases.

In this Special Issue, we aim to collect studies and reviews about the therapeutic landscape of MPNs, with a particular focus on new treatments.

Dr. Massimo Breccia
Dr. Francesca Palandri
Guest Editors

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 submissions that pass pre-check are 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 semimonthly 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 2900 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

  • JAK2 inhibitors
  • overall survival
  • new treatments
  • myeloproliferative neoplasms
  • MPNs

Published Papers (2 papers)

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Research

9 pages, 2292 KiB  
Communication
CALR but Not JAK2 Mutations Are Associated with an Overexpression of Retinoid X Receptor Alpha in Essential Thrombocythemia
by Ana Guijarro-Hernández, Cristina Hurtado, María José Larráyoz, María José Calasanz and José Luis Vizmanos
Cancers 2024, 16(8), 1511; https://doi.org/10.3390/cancers16081511 - 16 Apr 2024
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Abstract
Essential thrombocythemia (ET) is a blood cancer caused by mutations in JAK2 and CALR. It is widely recognized that both mutations lead to the constitutive activation of JAK2/STAT signaling, although other JAK/STAT-independent pathogenic mechanisms triggered by these alterations have also been described [...] Read more.
Essential thrombocythemia (ET) is a blood cancer caused by mutations in JAK2 and CALR. It is widely recognized that both mutations lead to the constitutive activation of JAK2/STAT signaling, although other JAK/STAT-independent pathogenic mechanisms triggered by these alterations have also been described in ET. In an attempt to study JAK2/STAT-independent mechanisms derived from CALR mutations, our research group created a C. elegans model with patient-like mutations in calreticulin that lacks JAK counterparts. The introduction of patient-like mutations in the calreticulin of C. elegans leads to an increase in the transcriptional expression of nhr-2, independently of JAK2/STAT activation. In the present study, we aim to verify if this mechanism is conserved in patients with ET harboring CALR mutations. To do so, we evaluated the expression of potential orthologs of nhr-2 in human cell lines of interest for the study, as well as in bone marrow (BM) or peripheral blood (PB) mononuclear cells from patients with CALR or JAK2 mutations. The results revealed that this mechanism is conserved in CALR-mutated ET patients, since CALR, but not JAK2 mutations, were associated with an overexpression of RXRA in patients with ET. The use of drugs targeting the activation or blockade of this target in the analyzed cell lines did not result in changes in cell viability. However, RXRA might be relevant in the disease, pointing to the need for future research testing retinoids and other drugs targeting RXRα for the treatment of ET patients. Full article
(This article belongs to the Special Issue Therapeutic Approaches for Myeloproliferative Neoplasms)
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15 pages, 1579 KiB  
Article
Predictors of Response to Hydroxyurea and Switch to Ruxolitinib in HU-Resistant Polycythaemia VERA Patients: A Real-World PV-NET Study
by Francesca Palandri, Elena Rossi, Giuseppe Auteri, Massimo Breccia, Simona Paglia, Giulia Benevolo, Elena M. Elli, Francesco Cavazzini, Gianni Binotto, Alessia Tieghi, Mario Tiribelli, Florian H. Heidel, Massimiliano Bonifacio, Novella Pugliese, Giovanni Caocci, Monica Crugnola, Francesco Mendicino, Alessandra D'Addio, Simona Tomassetti, Bruno Martino, Nicola Polverelli, Sara Ceglie, Camilla Mazzoni, Rikard Mullai, Alessia Ripamonti, Bruno Garibaldi, Fabrizio Pane, Antonio Cuneo, Mauro Krampera, Gianpietro Semenzato, Roberto M. Lemoli, Nicola Vianelli, Giuseppe A. Palumbo, Alessandro Andriani, Michele Cavo, Roberto Latagliata and Valerio De Stefanoadd Show full author list remove Hide full author list
Cancers 2023, 15(14), 3706; https://doi.org/10.3390/cancers15143706 - 21 Jul 2023
Cited by 1 | Viewed by 1222
Abstract
In polycythemia vera (PV), the prognostic relevance of an ELN-defined complete response (CR) to hydroxyurea (HU), the predictors of response, and patients’ triggers for switching to ruxolitinib are uncertain. In a real-world analysis, we evaluated the predictors of response, their impact on the [...] Read more.
In polycythemia vera (PV), the prognostic relevance of an ELN-defined complete response (CR) to hydroxyurea (HU), the predictors of response, and patients’ triggers for switching to ruxolitinib are uncertain. In a real-world analysis, we evaluated the predictors of response, their impact on the clinical outcomes of CR to HU, and the correlations between partial or no response (PR/NR) and a patient switching to ruxolitinib. Among 563 PV patients receiving HU for ≥12 months, 166 (29.5%) achieved CR, 264 achieved PR, and 133 achieved NR. In a multivariate analysis, the absence of splenomegaly (p = 0.03), pruritus (p = 0.002), and a median HU dose of ≥1 g/day (p < 0.001) remained associated with CR. Adverse events were more frequent with a median HU dose of ≥1 g/day. Overall, 283 PR/NR patients (71.3%) continued HU, and 114 switched to ruxolitinib. In the 449 patients receiving only HU, rates of thrombosis, hemorrhages, progression, and overall survival were comparable among the CR, PR, and NR groups. Many PV patients received underdosed HU, leading to lower CR and toxicity rates. In addition, many patients continued HU despite a PR/NR; however, splenomegaly and other symptoms were the main drivers of an early switch. Better HU management, standardization of the criteria for and timing of responses to HU, and adequate intervention in poor responders should be advised. Full article
(This article belongs to the Special Issue Therapeutic Approaches for Myeloproliferative Neoplasms)
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