Advancements and Challenges in Treating Chronic Myeloproliferative Neoplasms

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: 25 August 2024 | Viewed by 1940

Special Issue Editors


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Guest Editor
1. Department of Internal Medicine, General Hospital of Šibenik-Knin County, Šibenik, Croatia
2. Faculty of Medicine, University of Rijeka, Rijeka, Croatia
Interests: hematology; chronic myeloproliferative neoplasms; cardiovascular risk

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Co-Guest Editor
1. Pharmacy Department, General Hospital of Šibenik-Knin County, Šibenik, Croatia
2. Faculty of Medicine, University of Rijeka, Rijeka, Croatia
Interests: clinical trials

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Co-Guest Editor
1. Division of Hematology, University Hospital Rijeka, Rijeka, Croatia
2. Faculty of Medicine, University of Rijeka, Rijeka, Croatia
Interests: hematology; myeloproliferative neoplasms; stem cell transplantation; cellular therapy

Special Issue Information

Dear Colleagues,

This is an invitation to be part of a new Special Issue of the Journal of Clinical Medicine (JCM) entitled “Advancements and Challenges in Treating Chronic Myeloproliferative Neoplasms”. BCR::ABL1-negative chronic myeloproliferative neoplasms (MPNs), essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are disorders characterized by strong myeloproliferation, a chronic inflammatory state, the presence of various disease-related debilitating symptoms, high cardiovascular risk, and the intrinsic tendency to transform into secondary MF or acute leukemia. In recent years, significant advances in the MPN field have profoundly affected the diagnostic (i.e., novel molecular risk stratifications) and therapeutic landscapes (i.e., approval of ropeginterferon alfa-2b and the arrival of novel JAK inhibitors) in MPNs.

Therefore, the aim of this Special Issue is to give JCM readers an update on the diagnosis and the contemporary management of MPNs. In addition, manuscripts covering current controversies in the MPN field as well as original research articles providing novel concepts related to the pathophysiology, risk stratification, and management of MPNs are welcomed.

Dr. Ivan Krečak
Dr. Marko Skelin
Dr. Zinaida Perić
Guest Editors

Manuscript Submission Information

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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. Journal of Clinical Medicine 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 2600 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

  • chronic myeloproliferative neoplasms
  • thrombosis
  • survival
  • cardiovascular risk
  • treatment

Published Papers (2 papers)

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Research

11 pages, 4098 KiB  
Article
Does Double Mean Trouble? Coexistence of Myeloproliferative and Lymphoproliferative Neoplasms
by Danijela Lekovic, Jelena Ivanovic, Tatjana Terzic, Maja Perunicic Jovanovic, Marija Dencic Fekete, Jelica Jovanovic, Isidora Arsenovic, Vojin Vukovic, Jelena Bila, Andrija Bogdanovic and Darko Antic
J. Clin. Med. 2024, 13(6), 1816; https://doi.org/10.3390/jcm13061816 - 21 Mar 2024
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Abstract
Background: The occurrence of myeloproliferative neoplasms (MPNs) that evolve into each other is well-described, as is this occurrence of lymphoproliferative neoplasms (LPNs). However, less is known about rare MPN/LPN coexistence, and the aim of our study was to analyze charachteristics of these patients [...] Read more.
Background: The occurrence of myeloproliferative neoplasms (MPNs) that evolve into each other is well-described, as is this occurrence of lymphoproliferative neoplasms (LPNs). However, less is known about rare MPN/LPN coexistence, and the aim of our study was to analyze charachteristics of these patients after long term follow-up. Methods: Fourteen patients with MPN/LPN coexistence were diagnosed and treated according to guidelines at a single university center across two decades. Results: The overall median age was 53 years (22–69). MPNs patients with subsequent LPNs had a shorter period of second malignancy development and a more aggressive course of LPN, which can cause fatal outcomes. Polycythemia vera and chronic lymphocytic leukemia were most commonly associated (36%). The JAK2V617F mutation had 2/3 and cytogenetic abnormalities occurred in 1/3 of patients. MPN/LPN coexistence cases had significantly higher thrombotic potential (42.8%) and a higher third malignancy accruement frequency (21.4%) versus those without such malignancies. Conclusions: Considering the younger ages at MPN diagnosis, it is recommended to check regularly for blood lymphocytosis or lymphadenopathy occurrences and organomegaly progression faster than expected for MPN, with the aim of timely LPN diagnoses. The presence of molecular-cytogenetic abnormalities in a majority of patients indicate possible genetic instability and increased risk of development of multiple neoplasms, thus elevating thrombotic risk. Full article
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12 pages, 2185 KiB  
Article
e14a2 Transcript Favors Treatment-Free Remission in Chronic Myeloid Leukemia When Associated with Longer Treatment with Tyrosine Kinase Inhibitors and Sustained Deep Molecular Response
by Sílvia Marcé, Aleix Méndez, Blanca Xicoy, Natalia Estrada, Marta Cabezón, Antonella Luciana Sturla, Miriam Ratia García, Anna Angona, Paula Amat, Silvia Escribano Serrat, Emilia Scalzulli, Mireia Morgades, Alicia Senín, Juan Carlos Hernández-Boluda, Francisca Ferrer-Marín, Eduardo Anguita, Montserrat Cortés, Esther Plensa, Massimo Breccia, Valentín García-Gutierrez and Lurdes Zamoraadd Show full author list remove Hide full author list
J. Clin. Med. 2024, 13(3), 779; https://doi.org/10.3390/jcm13030779 - 29 Jan 2024
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Abstract
e13a2 and e14a2 are the most frequent transcript types of the BCR::ABL1 fusion gene in chronic myeloid leukemia (CML). The current goal with tyrosine kinase inhibitors (TKI) is to achieve sustained deep molecular response (DMR) in order to discontinue TKI treatment and remain [...] Read more.
e13a2 and e14a2 are the most frequent transcript types of the BCR::ABL1 fusion gene in chronic myeloid leukemia (CML). The current goal with tyrosine kinase inhibitors (TKI) is to achieve sustained deep molecular response (DMR) in order to discontinue TKI treatment and remain in the so-called treatment-free remission (TFR) phase, but biological factors associated with these goals are not well established. This study aimed to determine the effect of transcript type on TFR in patients receiving frontline treatment with imatinib (IM) or second-generation TKI (2G-TKI). Patients treated at least 119 months with IM presented less post-discontinuation relapse than those that discontinued IM before 119 months (p = 0.005). In addition, cases with the e14a2 transcript type treated at least 119 months with IM presented a better TFR (p = 0.024). On the other hand, the type of transcript did not affect the cytogenetic or molecular response in 2G-TKI treated patients; however, the use of 2G-TKI may be associated with higher and earlier DMR in patients with the e14a2 transcript. Full article
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