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Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives
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Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives

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

Deadline for manuscript submissions: 31 October 2025 | Viewed by 10905

Special Issue Editor

Prof. Dr. Arumugam Manoharan

E-Mail Website
Guest Editor
Faculty of Science, Medicine and Health, Graduate School of Medicine, University of Wollongong, Wollongong, NSW 2522, Australia
Interests: nutritional deficiency anaemias; myeloproliferative neoplasms; indolent/low-grade lymphoproliferative disorders; plasma cell dyscrasias

Special Issue Information

Dear Colleagues,

During the last two decades, our understanding of the pathogenesis of Ph-ve chronic myeloproliferative neoplasms.

(MPN: polycythemia vera, essential thrombocythemia and myelofibrosis) has increased significantly. Treatment of patients with MPN has also improved with the introduction of long-acting interferons for cytoreduction and use of JAK-2 inhibitor therapy for symptom relief in patients with myelofibrosis. Risk assessment and prevention of thrombosis have been the focus for several studies. Despite these advances, there are several areas of uncertainty in the management of MPN patients, e.g., dosage schedules for hydroxyurea therapy; indication for the use of aspirin for thromboprophylaxis; dosage schedules for aspirin prophylaxis; choice of JAK-2 inhibitor therapy and the use of cytoreductive therapy in conjunction with JAK-2 inhibitor therapy in patients with myelofibrosis.

The proposed Special Issue aims to record all the recent developments and current management practices, as well as outline possible areas of future research to improve clinical outcomes in patients with MPN.

Suggested Topics:

  • Pathogenesis of MPN and diagnosis in the era of molecular studies;
  • Risk assessment for thrombosis and initiation of thromboprophylaxis;
  • Prognosis and treatment of patients with polycythemia vera;
  • Prognosis and treatment of patients with essential thrombocythemia;
  • Prognosis and treatment of patients with myelofibrosis;
  • Long-term complications in patients with MPN.

Prof. Dr. Arumugam Manoharan
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 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. 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

  • diagnosis
  • pathogenesis
  • prognosis
  • thromboprophylaxis
  • treatment

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Published Papers (7 papers)

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Research

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10 pages, 899 KiB  
Article
Survey of Clinical Practice in Chronic Myeloproliferative Neoplasms in Croatia: A Study by the MPN Working Group Party of the Croatian Cooperative Group for Hematologic Diseases (KROHEM)
by Ivan Krecak, Marko Lucijanic and Rajko Kusec
J. Clin. Med. 2025, 14(5), 1524; https://doi.org/10.3390/jcm14051524 - 24 Feb 2025
Viewed by 436
Abstract
Background/Objectives: Chronic myeloproliferative neoplasms (MPNs), essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are hematopoietic stem cell disorders characterized by increased proliferation of mature myeloid cells, a chronic inflammatory state, and high cardiovascular risk. The diagnostic and therapeutic landscape in the [...] Read more.
Background/Objectives: Chronic myeloproliferative neoplasms (MPNs), essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are hematopoietic stem cell disorders characterized by increased proliferation of mature myeloid cells, a chronic inflammatory state, and high cardiovascular risk. The diagnostic and therapeutic landscape in the field of MPNs is rapidly evolving. Therefore, it is important to assess the behavior of physicians involved in the management of MPN patients to gain insight into how they embrace the current diagnostic and treatment landscape in real-life settings. Methods: An online anonymous survey consisting of 50 questions regarding their MPN practice and divided into four major domains (physician characteristics, diagnostic procedures, therapeutic decisions, and patient follow-up) was sent through the Croatian Cooperative Group for Hematologic Diseases’ (KROHEM’s) e-mailing list to all Croatian hematologists. Results: Thirty-one out of ninety adult hematologists (34.4% response rate) from KROHEM responded to this survey. There was a very high rate of self-proclaimed abidance to current international diagnostic and therapeutic recommendations, with no major differences among academic and community practices. However, some areas of uncertainty have been highlighted, especially in the frequency of cytogenetic and molecular testing, as well as very low implementation of the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) questionnaire in everyday practice. Conclusions: This study provides an important snapshot of the current MPN practice in Croatia. Similar studies from other countries are needed to provide a more detailed overview of real-life MPN practice globally. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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13 pages, 2444 KiB  
Article
Model-Assisted Spleen Contouring for Assessing Splenomegaly in Myelofibrosis: A Fast and Reproducible Approach to Evaluate Progression and Treatment Response
by Arman Sharbatdaran, Téa Cohen, Hreedi Dev, Usama Sattar, Vahid Bazojoo, Yin Wang, Zhongxiu Hu, Chenglin Zhu, Xinzi He, Dominick Romano, Joseph M. Scandura and Martin R. Prince
J. Clin. Med. 2025, 14(2), 443; https://doi.org/10.3390/jcm14020443 - 12 Jan 2025
Viewed by 1036
Abstract
Background/Objectives: Accurate and reproducible spleen volume measurements are essential for assessing treatment response and disease progression in myelofibrosis. This study evaluates techniques for measuring spleen volume on abdominal MRI. Methods: In 20 patients with bone marrow biopsy-proven myelofibrosis, 5 observers independently [...] Read more.
Background/Objectives: Accurate and reproducible spleen volume measurements are essential for assessing treatment response and disease progression in myelofibrosis. This study evaluates techniques for measuring spleen volume on abdominal MRI. Methods: In 20 patients with bone marrow biopsy-proven myelofibrosis, 5 observers independently measured spleen volume on 3 abdominal MRI pulse sequences, 3D-spoiled gradient echo T1, axial single-shot fast spin echo (SSFSE) T2, and coronal SSFSE T2, using ellipsoidal approximation, manual contouring, and 3D nnU-Net model-assisted contouring comparing coefficients of variation. Changes in spleen volume were compared to all information to assess which measurement technique tracked disease progression with the greatest accuracy. Results: The coefficient of variation in spleen volume measurements averaging over 3 sequences was significantly lower for model-assisted contouring, 1.6% and manual contouring, 3.5%, compared to ellipsoidal estimation from 3 dimensions measured on axial and coronal T2 images, 15, p < 0.001. In 4 subjects with divergent treatment response predictions, model-assisted contouring was consistent with all information while ellipsoidal estimation was not. Manual contouring tracked similarly to model-assisted contouring but required more operator time. Conclusions: Model-assisted segmentations provide efficient and more reproducible spleen volume measurements compared to estimates of spleen volume from ellipsoidal approximations and improve objective determinations of clinical trial enrollment eligibility based upon spleen volume as well as assessments of treatment response. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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9 pages, 1517 KiB  
Article
Translation, Cultural Adaptation, and Validation into Romanian of the Myeloproliferative Neoplasm Symptom Assessment Form—Total Symptom Score (MPN-SAF TSS or MPN-10) Questionnaire
by Mihnea-Alexandru Găman, Robyn Marie Scherber, Iulia Ursuleac, Ana Manuela Crişan, Sorina Nicoleta Bădeliţă, Bogdan Octavian Ionescu, Alexandra Elena Ghiaur, Melen Brînză, Nicoleta Pîrciulescu, Toma Octavian Lascăr, Camelia Cristina Diaconu, Amelia Maria Găman and Daniel Coriu
J. Clin. Med. 2024, 13(11), 3284; https://doi.org/10.3390/jcm13113284 - 2 Jun 2024
Viewed by 1694
Abstract
Background: Patients with myeloproliferative neoplasms (MPNs) experience a high disease-related symptom burden. A specific instrument to evaluate quality of life (QoL), i.e., the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN-10), was developed. We conducted the translation, cultural adaptation, and validation [...] Read more.
Background: Patients with myeloproliferative neoplasms (MPNs) experience a high disease-related symptom burden. A specific instrument to evaluate quality of life (QoL), i.e., the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN-10), was developed. We conducted the translation, cultural adaptation, and validation into Romanian of the MPN-10. Methods: We translated the MPN-10 and tested its psychometric properties. Results: We recruited 180 MPN patients: 66 polycythemia vera (36.67%), 61 essential thrombocythemia (33.89%), 51 primary and secondary myelofibrosis (SMF) (28.33%), and 2 MPN-unclassifiable (1.11%). The mean TSS was 19.51 ± 16.51 points. Fatigue, inactivity, and concentration problems were the most cumbersome symptoms. We detected scoring differences between MPN subtypes regarding weight loss (p < 0.001), fatigue (p = 0.006), early satiety (p = 0.007), night sweats (p = 0.047), pruritus (p = 0.05), and TSS (p = 0.021). There were strong positive associations between TSS and inactivity, fatigue, and concentration problems, and moderate negative correlations between QoL scores and all MPN-10 items. Cronbach’s α internal consistency coefficient was 0.855. The Kaiser–Meyer–Olkin construct validity test result was 0.870 and the Bartlett Sphericity Test was significant (p < 0.001). Symptom scores were loaded into one single factor according to the exploratory factor analysis. Conclusions: The Romanian MPN-10 version displayed excellent psychometric properties and is a reliable instrument for assessing symptom burden and QoL in Romanian MPN patients. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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Review

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17 pages, 707 KiB  
Review
Management of Bleeding, Thrombotic and Pregnancy-Related Complications in Women with Myeloproliferative Neoplasms: A Case-Based Review Focusing on Sex-Specific Challenges
by Thita Chiasakul and Ross I. Baker
J. Clin. Med. 2025, 14(5), 1537; https://doi.org/10.3390/jcm14051537 - 25 Feb 2025
Viewed by 566
Abstract
Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic disorders that pose unique challenges in women, particularly regarding thrombosis, bleeding, fertility, and pregnancy. Women with MPN exhibit distinct thrombotic and sometimes contradictory bleeding profiles, including a higher prevalence of unusual thrombosis such [...] Read more.
Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic disorders that pose unique challenges in women, particularly regarding thrombosis, bleeding, fertility, and pregnancy. Women with MPN exhibit distinct thrombotic and sometimes contradictory bleeding profiles, including a higher prevalence of unusual thrombosis such as cerebral and splanchnic vein thrombosis and increased risk of hemorrhage from anti-thrombotic medication, acquired von Willebrand syndrome and platelet dysfunction. Estrogen-containing contraceptives should generally be avoided due to thrombotic risk. Around 10–20% of newly diagnosed MPN cases are women of childbearing age and the number is increasing annually. MPN patients when compared to controls have a lower rate of live birth rate of 71% vs. 80% with a hazard ratio of 0.78 (95% CI: 0.68–0.90), and increased preterm birth (14% vs. 4%), low birth weight (<2500 g, 10% vs. 4%), and increased cesarean section rate (32% vs. 17%). Management of MPN-related pregnancy requires specific considerations regarding the prevention of thrombosis, bleeding, and pregnancy-related complications. Management strategies during pregnancy include low-dose aspirin and consideration of low-molecular-weight heparin and interferon. Despite these challenges, most women with MPN can achieve successful pregnancies with optimized care. In this case-based review, we present two cases that illustrate key aspects of managing MPN in women, summarize the current literature, and propose a diagnostic and management framework tailored to these complexities. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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26 pages, 1685 KiB  
Review
Myeloproliferative Neoplasms: Challenging Dogma
by Jerry L. Spivak
J. Clin. Med. 2024, 13(22), 6957; https://doi.org/10.3390/jcm13226957 - 19 Nov 2024
Cited by 1 | Viewed by 1957
Abstract
Myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis, and primary myelofibrosis are a unique group of clonal hematopoietic stem cell neoplasms that share somatic, gain-in-function driver mutations in JAK2, CALR, and MPL. As a consequence, these disorders exhibit similar phenotypic features, the [...] Read more.
Myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis, and primary myelofibrosis are a unique group of clonal hematopoietic stem cell neoplasms that share somatic, gain-in-function driver mutations in JAK2, CALR, and MPL. As a consequence, these disorders exhibit similar phenotypic features, the most common of which are the ceaseless production of normal erythrocytes, myeloid cells, platelets alone or in combination, extramedullary hematopoiesis, myelofibrosis, and a potential for leukemic transformation. In the case of polycythemia vera and essential thrombocytosis, however, prolonged survival is possible. With an incidence value in the range of 0.5–2.0/100,000, myeloproliferative neoplasms are rare disorders, but they are not new disorders, and after a century of scrutiny, their clinical features and natural histories are well-defined, though their individual management continues to be controversial. With respect to polycythemia vera, there has been a long-standing dispute between those who believe that the suppression of red blood cell production by chemotherapy is superior to phlebotomy to prevent thrombosis, and those who do not. With respect to essential thrombocytosis, there is a similar dispute about the role of platelets in veinous thrombosis, and the role of chemotherapy in preventing thrombosis by suppressing platelet production. Linked to these disputes is another: whether therapy with hydroxyurea promotes acute leukemia in disorders with a substantial possibility of longevity. The 21st century revealed new insights into myeloproliferative neoplasms with the discovery of their three somatic, gain-of-function driver mutations. Almost immediately, this triggered changes in the diagnostic criteria for myeloproliferative neoplasms and their therapy. Most of these changes, however, conflicted with prior well-validated, phenotypically driven diagnostic criteria and the management of these disorders. The aim of this review is to examine these conflicts and demonstrate how genomic discoveries in myeloproliferative neoplasms can be used to effectively complement the known phenotypic features of these disorders for their diagnosis and management. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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13 pages, 757 KiB  
Review
Thrombotic, Cardiovascular, and Microvascular Complications of Myeloproliferative Neoplasms and Clonal Hematopoiesis (CHIP): A Narrative Review
by Andrew I. Schafer and Douglas L. Mann
J. Clin. Med. 2024, 13(20), 6084; https://doi.org/10.3390/jcm13206084 - 12 Oct 2024
Cited by 2 | Viewed by 1305
Abstract
The most common causes of morbidity and mortality in the myeloproliferative neoplasms (MPNs), with the exception of myelofibrosis, are venous and arterial thrombosis, as well as more recently discovered cardiovascular disease (CVD). Clonal hematopoiesis of indeterminate potential (CHIP) is the subclinical finding in [...] Read more.
The most common causes of morbidity and mortality in the myeloproliferative neoplasms (MPNs), with the exception of myelofibrosis, are venous and arterial thrombosis, as well as more recently discovered cardiovascular disease (CVD). Clonal hematopoiesis of indeterminate potential (CHIP) is the subclinical finding in an individual of somatic mutations that are also found in clinically overt MPNs and other myeloid malignancies. The prevalence of “silent” CHIP increases with age. CHIP can transform into a clinically overt MPN at an estimated rate of 0.5 to 1% per year. It is likely, therefore, but not proven, that many, if not all, MPN patients had antecedent CHIP, possibly for many years. Moreover, both individuals with asymptomatic CHIP, as well as clinically diagnosed patients with MPN, can develop thrombotic complications. An unexpected and remarkable discovery during the last few years is that even CHIP (as well as MPNs) are significant, independent risk factors for CVD. This review discusses up-to-date information on the types of thrombotic and cardiovascular complications that are found in CHIP and MPN patients. A systemic inflammatory state (that is often subclinical) is most likely to be a major mediator of adverse reciprocal bone marrow–cardiovascular interplay that may fuel the development of progression of MPNs, including its thrombotic and vascular complications, as well as the worsening of cardiovascular disease, possibly in a “vicious cycle”. Translating this to clinical practice for hematologists and oncologists who treat MPN patients, attention should now be paid to ensuring that cardiovascular risk factors are controlled and minimized, either by the patient’s cardiologist or primary care physician or by the hematologist/oncologist herself or himself. This review is intended to cover the clinical aspects of thrombosis and cardiovascular complications in the MPN, accompanied by pathobiological comments. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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15 pages, 442 KiB  
Review
Impact of Phlebotomy on Quality of Life in Low-Risk Polycythemia Vera
by Nathan Visweshwar, Bradley Fletcher, Michael Jaglal, Damian A. Laber, Ankita Patel, Jennifer Eatrides, Geetha Rajasekharan Rathnakumar, Keshav Visweswaran Iyer, Irmel Ayala and Arumugam Manoharan
J. Clin. Med. 2024, 13(16), 4952; https://doi.org/10.3390/jcm13164952 - 22 Aug 2024
Cited by 1 | Viewed by 3036
Abstract
Polycythemia vera is an indolent myeloproliferative disorder that predisposes patients to venous and arterial thrombosis and can transform into myelofibrosis and acute myeloid leukemia. Consistent phlebotomy prevents life-threatening cerebrovascular and coronary artery disease and prolongs survival in low-risk polycythemia vera (patients under 60 [...] Read more.
Polycythemia vera is an indolent myeloproliferative disorder that predisposes patients to venous and arterial thrombosis and can transform into myelofibrosis and acute myeloid leukemia. Consistent phlebotomy prevents life-threatening cerebrovascular and coronary artery disease and prolongs survival in low-risk polycythemia vera (patients under 60 years without thrombosis). However, despite its effectiveness in preventing serious complications, phlebotomy does not necessarily enhance the quality of life (QoL). This review assesses QoL issues associated with low-risk PV, explores alternative management strategies such as erythrocytapheresis, and discusses the roles of hydroxyurea, peginterferon, ruxolitinib, and other novel agents in potentially improving disease management and patient outcomes. Full article
(This article belongs to the Special Issue Myeloproliferative Neoplasms (MPN): Recent Advances, Current Practices and Future Perspectives)
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