RESUMEN

Treatment selection for patients with polycythemia vera (PV) is based on patient age and history of thrombosis. The standard treatment is low-dose aspirin and phlebotomy for low-risk PV, with cytoreductive therapy added for high-risk PV. Thrombotic events and disease progression due to PV clone expansion affect the prognosis of PV. Although phlebotomy is effective in controlling hematocrit level, it has no effect on disease progression or PV-related symptoms. In Western countries, interferon (IFN) has been used as a cytoreductive therapy for PV. Long-term IFN therapy has been shown to result in sustained hematologic remission and molecular responses. Ropeginterferon-α-2b (ropeg-IFN), which is administered every two weeks, has recently become available. Clinical trials in patients with PV have shown that ropeg-IFN treatment is safe and efficacious, reducing JAK2V617F allele burden. Ropeg-IFN could ultimately affect long-term hematologic remission and molecular response in younger patients with low-risk PV, and may even offer a cure.

Asunto(s)

Policitemia Vera , Policitemia Vera/terapia , Humanos , Janus Quinasa 2/genética , Interferón-alfa/uso terapéutico , Interferón-alfa/administración & dosificación , Factores de Riesgo

RESUMEN

Serum erythropoietin (sEPO) is an initial screening tool for distinguishing polycythemia vera (PV) from secondary erythrocytosis (SE), but defining 'subnormal' sEPO levels for PV diagnosis remains contentious, complicating its clinical utility. This study compares the diagnostic performance of sEPO across established subnormal limits, including reference interval (RI), clinical decision limit (CDL), and functional reference limit. sEPO levels were analyzed in 393 healthy donors (HDs) and 90 patients (41 PV and 49 SE), who underwent bone marrow biopsy and genetic tests due to erythrocytosis. The RI (2.5-97.5 percentile from HDs) of sEPO was 5.3-26.3 IU/L. A CDL of 3.1 IU/L, determined by ROC analysis in erythrocytosis patients, had a sensitivity of 80.5% and specificity of 87.8% for diagnosing PV. A functional reference limit of 7.0 IU/L, estimated based on the relationship between sEPO and hemoglobin, hematocrit, and WBC, increased sensitivity to 97.6% but decreased specificity to 46.7%. Using 5.3 IU/L as a 'subnormal' limit identified all three JAK2-negative PV cases, increasing the sensitivity and negative predictive value to 97.6% and 97.0%, respectively. Combining the RI, CDL, and functional reference limit may improve PV diagnostic accuracy.

RESUMEN

Extramedullary hematopoiesis (EMH) is the formation of blood cells outside the bone marrow, typically occurring in response to chronic anemia or bone marrow dysfunction. While EMH is most commonly observed in the liver, spleen, and lymph nodes, its occurrence in the kidney is exceedingly rare. In this case report, we are presenting a case of a 49-year-old male diagnosed with polycythemia vera who had an incidental right renal mass, which was histo-pathologically proven as extramedullary hematopoiesis in the right kidney mimicking lymphoma. This case underscores the importance of considering EMH in the differential diagnosis of renal masses, especially in patients with a history of myeloproliferative disorders. Early recognition and appropriate management are crucial to avoid unnecessary interventions and manage the underlying hematological condition effectively. Accurate diagnosis through histopathological examination is crucial to avoid unnecessary surgical interventions.

RESUMEN

The coronavirus disease 2019 (COVID-19) pandemic has significantly impacted the diagnosis and management of tuberculosis (TB), a major public health issue. This case report discusses a 70-year-old female with post-polycythemia vera myelofibrosis (post-PV MF) treated with ruxolitinib who developed miliary TB amidst a COVID-19 infection. The patient presented with a flu-like syndrome over the past week with fatigue and weight loss the last month. When she was admitted to the hospital, the real-time polymerase chain reaction (RT-PCR) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was positive. Despite the typical COVID-19 presentation, her clinical and radiographic features raised suspicion for disseminated TB. Diagnostic tests, including bronchoscopy and PCR for Mycobacterium tuberculosis, confirmed miliary TB. She was treated with a standard antitubercular regimen, leading to symptomatic improvement. The interplay between COVID-19 and TB is complex, with COVID-19-induced immunosuppression, particularly lymphocytopenia, facilitating TB reactivation. Additionally, ruxolitinib, a Janus kinase (JAK) inhibitor used for myelofibrosis, impairs immune defense mechanisms, increasing infection risk, including TB. Prompt and accurate diagnosis of TB in the context of COVID-19 is crucial for effective management and improved patient outcomes. Clinicians should remain vigilant for TB reactivation in patients undergoing treatments such as ruxolitinib and consider alternative diagnoses despite positive SARS-CoV-2 tests. This report highlights the necessity for a comprehensive evaluation and timely intervention to mitigate the compounded risks of COVID-19 and TB.

RESUMEN

Background: Polycythemia vera (PV) is a myeloproliferative disease characterized by significantly higher hemoglobin levels and positivity for JAK2 mutation. Thrombosis is the main risk event of this disease. Atherosclerosis (AS) can markedly increase the risk of arterial thrombosis in patients with PV. The objectives of our study were to identify potential biomarkers for PV-related AS and to explore the molecular biological association between PV and AS. Methods: We extracted microarray datasets from the Gene Expression Omnibus (GEO) dataset for PV and AS. Common differentially expressed genes (CGs) were identified by differential expression analysis. Functional enrichment and protein-protein interaction (PPI) networks were constructed from the CG by random forest models using LASSO regression to identify pathogenic genes and their underlying processes in PV-related AS. The expression of potential biomarkers was validated using an external dataset. A diagnostic nomogram was constructed based on potential biomarkers to predict PV-related AS, and its diagnostic performance was assessed using ROC, calibration, and decision curve analyses. Subsequently, we used single-cell gene set enrichment analysis (GSEA) to analyze the immune signaling pathways associated with potential biomarkers. We also performed immune infiltration analysis of AS with "CIBERSORT" and calculated Pearson's correlation coefficients for potential biomarkers and infiltrating immune cells. Finally, we observed the expression of potential biomarkers in immune cells based on the single-cell RNA dataset. Results: Fifty-two CGs were identified based on the intersection between up-regulated and down-regulated genes in PV and AS. Most biological processes associated with CGs were cytokines and factors associated with chemotaxis of immune cells. The PPI analysis identified ten hub genes, and of these, CCR1 and MMP9 were selected as potential biomarkers with which to construct a diagnostic model using machine learning methods and external dataset validation. These biomarkers could regulate Toll-like signaling, NOD-like signaling, and chemokine signaling pathways associated with AS. Finally, we determined that these potential biomarkers had a strong correlation with macrophage M0 infiltration. Further, the potential biomarkers were highly expressed in macrophages from patients with AS. Conclusion: We identified two CGs (CCR1 and MMP9) as potential biomarkers for PV-related AS and established a diagnostic model based on them. These results may provide insight for future experimental studies for the diagnosis and treatment of PV-related AS.

RESUMEN

Introduction: The Janus kinase (JAK) family includes four cytoplasmic tyrosine kinases (JAK1, JAK2, JAK3, and TYK2) constitutively bound to several cytokine receptors. JAKs phosphorylate downstream signal transducers and activators of transcription (STAT). JAK-STAT5 pathways play a critical role in basophil and mast cell activation. Previous studies have demonstrated that inhibitors of JAK-STAT pathway blocked the activation of mast cells and basophils. Methods: In this study, we investigated the in vitro effects of ruxolitinib, a JAK1/2 inhibitor, on IgE- and IL-3-mediated release of mediators from human basophils, as well as substance P-induced mediator release from skin mast cells (HSMCs). Results: Ruxolitinib concentration-dependently inhibited IgE-mediated release of preformed (histamine) and de novo synthesized mediators (leukotriene C4) from human basophils. Ruxolitinib also inhibited anti-IgE- and IL-3-mediated cytokine (IL-4 and IL-13) release from basophils, as well as the secretion of preformed mediators (histamine, tryptase, and chymase) from substance P-activated HSMCs. Discussion: These results indicate that ruxolitinib, inhibiting the release of several mediators from human basophils and mast cells, is a potential candidate for the treatment of inflammatory disorders.

Asunto(s)

Basófilos , Janus Quinasa 1 , Janus Quinasa 2 , Mastocitos , Nitrilos , Pirazoles , Pirimidinas , Humanos , Basófilos/efectos de los fármacos , Basófilos/inmunología , Basófilos/metabolismo , Pirimidinas/farmacología , Nitrilos/farmacología , Mastocitos/efectos de los fármacos , Mastocitos/inmunología , Mastocitos/metabolismo , Pirazoles/farmacología , Janus Quinasa 1/antagonistas & inhibidores , Janus Quinasa 1/metabolismo , Janus Quinasa 2/metabolismo , Janus Quinasa 2/antagonistas & inhibidores , Células Cultivadas , Inhibidores de las Cinasas Janus/farmacología , Citocinas/metabolismo , Inmunoglobulina E/inmunología , Inmunoglobulina E/metabolismo , Inhibidores de Proteínas Quinasas/farmacología

RESUMEN

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.

RESUMEN

BACKGROUND: Woven coronary artery (WCA) is a rare and underdiagnosed congenital anomaly that involves multiple thin and tortuous epicardial arterial conduits reassembling distally into a single lumen. Recanalized thrombus may present as woven-like coronary arteries, appearing similar to WCA on angiographic images. CASE PRESENTATION: A 58-year-old female patient with intermittent chest pain for 5 years and polycythaemia vera (PV) for 8 years. The left anterior descending artery was presented like WCA on coronary angiography and finally confirmed as recanalized thrombus by optical coherence tomography(OCT), which might have been caused by PV. Given the patient's high thrombotic risk of PV and thrombotic changes in the left circumflex artery (LCX), we ultimately chose a conservative treatment without stenting. CONCLUSIONS: OCT would be needed for the diagnosis and differential diagnosis of woven-like coronary arteries. And physicians should take an appropriate treatment in a personalized way in patients with PV.

Asunto(s)

Angiografía Coronaria , Trombosis Coronaria , Anomalías de los Vasos Coronarios , Policitemia Vera , Valor Predictivo de las Pruebas , Tomografía de Coherencia Óptica , Humanos , Femenino , Persona de Mediana Edad , Policitemia Vera/complicaciones , Policitemia Vera/diagnóstico , Policitemia Vera/terapia , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/complicaciones , Anomalías de los Vasos Coronarios/terapia , Trombosis Coronaria/diagnóstico por imagen , Trombosis Coronaria/terapia , Resultado del Tratamiento , Tratamiento Conservador , Vasos Coronarios/diagnóstico por imagen

RESUMEN

Myeloproliferative neoplasms (MPNs) are a group of rare chronic progressive blood cancers that vary widely in clinical presentation, yet all patients have a risk of disease progression and thrombotic complications. Diseases include primary myelofibrosis, polycythemia vera, and essential thrombocythemia. With current treatment approaches, most patients live a prolonged life, but many experience a complex of symptoms that negatively influence their functional status and quality of life. Although significant advances have been made in preventing arterial and venous complications while mitigating inflammatory processes, comprehensive palliative care can help address unmet complex physical and psychosocial needs on a long-term basis. This article, created by a multidisciplinary group of providers, offers an overview of MPNs so palliative care clinicians can better support patients with these hematologic cancers.

Asunto(s)

Resistencia a Medicamentos , Hidroxiurea , Policitemia Vera , Humanos , Hidroxiurea/uso terapéutico , Policitemia Vera/tratamiento farmacológico

RESUMEN

Myeloproliferative neoplasms (MPNs), encompassing disorders like polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are characterized by clonal hematopoiesis without the Philadelphia chromosome. The JAK2 V617F mutation is prevalent in PV, ET, and PMF, while mutations in MPL and CALR also play significant roles. These conditions predispose patients to thrombotic events, with PMF exhibiting the lowest survival among MPNs. Chronic inflammation, driven by cytokine release from aberrant leukocytes and platelets, amplifies cardiovascular risk through various mechanisms, including atherosclerosis and vascular remodeling. Additionally, MPN-related complications like pulmonary hypertension and cardiac fibrosis contribute to cardiovascular morbidity and mortality. This review consolidates recent research on MPNs' cardiovascular implications, emphasizing thrombotic risk, chronic inflammation, and vascular stiffness. Understanding these associations is crucial for developing targeted therapies and improving outcomes in MPN patients.

RESUMEN

INTRODUCTION: The treatment landscape of polycythemia vera (PV) has seen major advancements within the last decade including approval of ruxolitinib in the second line setting after hydroxyurea, ropegylated interferon-α2b, and advanced clinical development of a novel class of agents called hepcidin mimetics. AREAS COVERED: We provide a comprehensive review of the evidence discussing the risk stratification, treatment indications, role and limitations of phlebotomy only approach and pivotal trials covering nuances related to the use of interferon-α (IFN-α), ruxolitinib, hepcidin mimetics, and upcoming investigational agents including HDAC and LSD1 inhibitors. EXPERT OPINION: The research paradigm in PV is slowly shifting from the sole focus on hematocrit control and moving toward disease modification. The discovery of hepcidin mimetics has come as a breakthrough in restoring iron homeostasis, achieving phlebotomy-independence and may lead to improved thrombosis-free survival with stricter hematocrit control. On the other hand, emerging data with IFN- α and ruxolitinib as well as combination of the two agents suggests the potential for achieving molecular remission in a subset of PV patients and long-term follow-up is awaited to validate the correlation of molecular responses with clinically relevant outcomes of progression-free and thrombosis-free survival.

Asunto(s)

Interferón-alfa , Nitrilos , Flebotomía , Policitemia Vera , Pirazoles , Policitemia Vera/tratamiento farmacológico , Humanos , Nitrilos/uso terapéutico , Pirazoles/uso terapéutico , Interferón-alfa/uso terapéutico , Pirimidinas/uso terapéutico , Hepcidinas/metabolismo , Interferón alfa-2/uso terapéutico , Hematócrito

RESUMEN

INTRODUCTION: The hallmark discovery of hyperactivation of the janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway was a sentinel moment in the history of myeloproliferative neoplasms (MPNs). This finding paved the way for the development of JAK inhibitors, which now represent the foundation of myelofibrosis therapy. With four JAK inhibitors now approved for myelofibrosis, awareness of their clinical efficacy and safety data and recognition of their unique pharmacologic attributes are of critical importance. Additionally, ruxolitinib represents an integral part of the therapeutic arsenal for polycythemia vera. AREAS COVERED: This review provides a broad overview of the published literature supporting JAK inhibitor therapy for MPNs. Primarily focusing on myelofibrosis, each of the four available JAK inhibitors is reviewed in detail, including pharmacology, efficacy, and safety data. Failure of JAK inhibitors and future directions in JAK inhibitor therapy are also discussed. EXPERT OPINION: JAK inhibitors revolutionized the treatment of MPNs and have dramatically improved patient outcomes. However, data informing selection between currently available JAK inhibitors is limited. These agents are not curative and eventually fail most patients with myelofibrosis. Combining JAK inhibitors with novel targeted agents appears to be the most promising path to further improve outcomes.

Asunto(s)

Inhibidores de las Cinasas Janus , Trastornos Mieloproliferativos , Mielofibrosis Primaria , Humanos , Inhibidores de las Cinasas Janus/uso terapéutico , Inhibidores de las Cinasas Janus/farmacología , Trastornos Mieloproliferativos/tratamiento farmacológico , Mielofibrosis Primaria/tratamiento farmacológico , Quinasas Janus/antagonistas & inhibidores , Nitrilos/uso terapéutico , Animales , Desarrollo de Medicamentos , Antineoplásicos/uso terapéutico , Antineoplásicos/farmacología , Pirazoles/uso terapéutico , Pirazoles/farmacología , Policitemia Vera/tratamiento farmacológico , Policitemia Vera/genética , Pirimidinas/uso terapéutico , Pirimidinas/farmacología

RESUMEN

Polycythemia vera (PV) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by clonal erythrocytosis. A phase 2 study reported that ropeginterferon alfa-2b is a well-tolerated and effective treatment for PV in Japanese patients. This post hoc analysis of the phase 2 data further evaluated outcomes in patients at low risk of thrombosis (low-risk PV). Among 20 patients with low-risk PV, 60.0% (12/20) and 85.0% (17/20) achieved < 45% hematocrit by weeks 24 and 52, respectively. The proportion of responders with complete hematologic response (CHR) was 60.0% (12/20) at week 52, and the median time to response was 11.9 months. The mean JAK2 V617F allele burden decreased from 75.8% at baseline to 53.7% at week 52. No patient experienced thrombosis or bleeding episodes. All patients experienced treatment-emergent adverse events (TEAEs) related to ropeginterferon alfa-2b, but no grade ≥ 3 TEAEs or deaths related to ropeginterferon alfa-2b occurred, and no new safety concerns arose. This analysis indicated that ropeginterferon alfa-2b may be an effective treatment option for Japanese patients with low-risk PV.

Asunto(s)

Interferón alfa-2 , Interferón-alfa , Policitemia Vera , Polietilenglicoles , Proteínas Recombinantes , Humanos , Policitemia Vera/tratamiento farmacológico , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/uso terapéutico , Proteínas Recombinantes/efectos adversos , Interferón-alfa/uso terapéutico , Interferón-alfa/efectos adversos , Interferón-alfa/administración & dosificación , Polietilenglicoles/efectos adversos , Polietilenglicoles/administración & dosificación , Polietilenglicoles/uso terapéutico , Interferón alfa-2/uso terapéutico , Interferón alfa-2/administración & dosificación , Interferón alfa-2/efectos adversos , Masculino , Persona de Mediana Edad , Femenino , Resultado del Tratamiento , Anciano , Janus Quinasa 2/genética , Japón , Adulto , Pueblo Asiatico , Pueblos del Este de Asia

RESUMEN

BACKGROUND: Here the authors present the case of a 43-year-old male with a history of T-cell lymphoma, which was treated with azacitidine plus cyclophosphamide, doxorubicin, vincristine, and prednisone and autologous hematopoietic cell transplant, and high-risk polycythemia vera (PCV) presenting with severe lower-back pain radiating to the bilateral legs with associated lower-extremity weakness and splenomegaly. OBSERVATIONS: T2-weighted magnetic resonance imaging revealed multilevel epidural lesions involving T1-10 and S1-2. Because of severe spinal canal stenosis, the patient underwent surgical decompression of T5-7, with immediate postoperative alleviation of the lower-extremity pain and complete resolution of the lower-leg weakness. Biopsy results revealed extramedullary hematopoiesis (EMH) mimicking a spinal epidural tumor. EMH is radiosensitive and displays a rapid response to low dosages, so the patient was further treated with palliative radiation therapy for residual tumors and symptom alleviation, as well as hydroxyurea and corticosteroids as indicated for cytoreduction. LESSONS: EMH associated with PCV or myeloproliferative conditions occurring within the spine is a rare phenomenon without a standard treatment approach. https://thejns.org/doi/10.3171/CASE23659.

RESUMEN

This research provides a comprehensive bibliometric analysis of polycythemia vera (PV) research trends, encompassing data from 1969 to 2024. Utilizing advanced tools, key findings reveal a notable increase in scientific production over time, reflecting growing interest and investment in PV research. Prominent themes include genetic studies, targeted therapies, and precision medicine approaches. The analysis identifies leading authors, institutions, and countries contributing to PV research, highlighting the importance of global collaboration. The study emphasizes the need to broaden genetic investigations, explore the bone marrow microenvironment, and enhance precision medicine strategies. The implications of this research extend to clinical practice, with potential advancements in diagnostics, treatments, and patient outcomes. Ultimately, addressing these challenges and embracing emerging opportunities can propel PV research forward, fostering innovation and improving the lives of affected individuals.

RESUMEN

The study utilized Fourier transform infrared (FTIR) spectroscopy coupled with chemometrics to investigate protein composition and structural changes in the blood serum of patients with polycythemia vera (PV). Principal component analysis (PCA) revealed distinct biochemical properties, highlighting elevated absorbance of phospholipids, amides, and lipids in PV patients compared to healthy controls. Ratios of amide I/amide II and amide I/amide III indicated alterations in protein structures. Support vector machine analysis and receiver operating characteristic curves identified amide I as a crucial predictor of PV, achieving 100% accuracy, sensitivity, and specificity, while amide III showed a lower predictive value (70%). PCA analysis demonstrated effective differentiation between PV patients and controls, with key wavenumbers including amide II, amide I, and CH lipid vibrations. These findings underscore the potential of FTIR spectroscopy for diagnosing and monitoring PV.

Asunto(s)

Amidas , Biomarcadores , Policitemia Vera , Análisis de Componente Principal , Espectroscopía Infrarroja por Transformada de Fourier , Humanos , Policitemia Vera/sangre , Policitemia Vera/diagnóstico , Biomarcadores/sangre , Persona de Mediana Edad , Femenino , Masculino , Anciano , Máquina de Vectores de Soporte , Estudios de Casos y Controles , Adulto

RESUMEN

Thrombosis in myeloproliferative neoplasms (MPNs) is an important clinical problem, and risk-stratified management is essential. To identify the clinical characteristics of thrombosis in patients with MPNs, a nationwide multi-institutional retrospective analysis (JSH-MPN-R18) was conducted. The aim of the present study was to perform a sub-analysis of JSH-MPN-R18 findings to clarify the predictive parameters for thrombosis among complete blood count (CBC) results. Among the patients enrolled in JSH-MPN-R18, those with essential thrombocythemia (ET; n = 1152) and polycythemia vera (PV; n = 456) were investigated. We analyzed and compared CBC parameters between patients with and those without any thrombotic events using Welch's T-test. Statistical analyses were performed using the R statistical software. Thrombotic events were observed in 74 patients with ET. In multivariate analysis, only the neutrophil ratio was slightly but significantly higher for ET patients with thrombosis than for those without (p < 0.05). Of note, the absolute neutrophil count (aNeu) was considered a useful predictive tool for thrombosis among patients classified as low-risk according to the revised International Prognostic Score of Thrombosis for Essential Thrombocythemia. Among PV patients, those with thrombosis showed significantly higher hematocrit and aNeu than did those without thrombosis. As a thrombosis-associated factor, the neutrophil ratio was slightly but significantly elevated in patients with ET. This myeloid skew might reflect a higher value of JAK2 V617F allelic frequency in patients with ET with thrombosis; this was not clarified in JSH-MPN-R18. Further accumulation of evidence, including genetic information for JAK2 and other passenger mutations, is warranted.

Asunto(s)

Janus Quinasa 2 , Neutrófilos , Policitemia Vera , Trombocitemia Esencial , Trombosis , Humanos , Trombosis/etiología , Trombosis/sangre , Femenino , Masculino , Persona de Mediana Edad , Janus Quinasa 2/genética , Anciano , Estudios Retrospectivos , Trombocitemia Esencial/sangre , Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/genética , Policitemia Vera/sangre , Policitemia Vera/complicaciones , Policitemia Vera/genética , Adulto , Trastornos Mieloproliferativos/sangre , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/genética , Recuento de Leucocitos , Valor Predictivo de las Pruebas , Anciano de 80 o más Años

Asunto(s)

Interferón alfa-2 , Interferón-alfa , Policitemia Vera , Polietilenglicoles , Proteínas Recombinantes , Adulto , Humanos , Aberraciones Cromosómicas , Interferón alfa-2/uso terapéutico , Interferón-alfa/uso terapéutico , Policitemia Vera/tratamiento farmacológico , Policitemia Vera/genética , Polietilenglicoles/uso terapéutico , Proteínas Recombinantes/uso terapéutico

RESUMEN

Myeloproliferative neoplasms (MPNs) occur due to the abnormal proliferation of one or more terminal myeloid cell lines in peripheral blood. Subjects suffering from MPNs display a high burden of cardiovascular risk factors, and thrombotic events are often the cause of death in this population of patients. Herein, we provide a brief overview of dyslipidemia and metabolic syndrome and their epidemiology in MPNs and examine the common molecular mechanisms between dyslipidemia, metabolic syndrome, and MPNs, with a special focus on cardiovascular risk, atherosclerosis, and thrombotic events. Furthermore, we investigate the impact of dyslipidemia and metabolic syndrome on the occurrence and survival of thrombosis in MPN patients, as well as the management of dyslipidemia in MPNs, and the impact of MPN treatment on serum lipid concentrations, particularly as side/adverse effects reported in the context of clinical trials.