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Cardiovascular Risk in Philadelphia-Negative Myeloproliferative Neoplasms: Mechanisms and Implications-A Narrative Review.
Curr Issues Mol Biol
August 2024
Faculty of Medicine, Lucian Blaga University of Sibiu, 550169 Sibiu, Romania.
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.
View Article and Find Full Text PDFChromosome 9p trisomy increases stem cells clonogenic potential and fosters T-cell exhaustion in JAK2-mutant myeloproliferative neoplasms.
Leukemia
October 2024
Interdepartmental Centre for Stem Cells and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy.
Article Synopsis
- JAK2V617F is the most common genetic mutation in Philadelphia-negative chronic Myeloproliferative Neoplasms (MPNs), and researchers believe abnormalities in Chromosome 9 may influence the disease in patients with this mutation.
- A specific group of MPN patients, called +9p patients, were found to have three copies of the JAK2 gene and nearby genes, leading to increased production of the immunosuppressive PD-L1 protein.
- The study showed that these +9p patients have a distinct cancer profile, characterized by greater stem cell-like properties and an immune response that results in exhausted T cells, highlighting a complex interaction between +9p and JAK2V617F mutations.
Myeloproliferative neoplasms: young patients, current data and future considerations.
Ann Hematol
September 2024
Department of Hematology, Guy's and St Thomas' National Health Service Foundation Trust, London, UK.
Article Synopsis
- Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) primarily affect older adults, with fewer cases seen in children and young adults, leading to less knowledge about their symptoms and treatment in these younger demographics.
- Diagnosis in younger patients can be challenging due to the rarity of specific genetic mutations and differences in blood counts compared to adults.
- Effective management of young MPN patients is complex due to their unique needs and longer life expectancies, highlighting the necessity for collaboration between pediatricians and adult hematologists to develop better treatment protocols.
Retinal Vessel Analysis and Microvascular Abnormalities in Patients with Philadelphia-Negative Chronic Myeloproliferative Neoplasms.
J Clin Med
April 2024
Hematology Unit, Department of Human Pathology in Adulthood and Childhood "Gaetano Barresi", University of Messina, Via Consolare Valeria, 98125 Messina, Italy.
: Philadelphia-negative chronic myeloproliferative neoplasms are a group of clonal hematopoietic disorders including polycythemia vera, essential thrombocythemia, and primary myelofi-brosis. These neoplasms are characterized by an increased risk of thrombotic complications. Several studies have highlighted that the study of vessels of the retina offers the opportunity to visualize, in vivo, the damage to microcirculation that is common in various systemic pathologies.
View Article and Find Full Text PDFRisk of hepatitis B reactivation in patients with myeloproliferative neoplasms treated with ruxolitinib.
World J Hepatol
November 2023
Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, Bucharest 050474, Romania.
Classical Philadelphia-negative myeloproliferative neoplasms (MPNs), , polycythemia vera, essential thrombocythemia, and primary/secondary myelofibrosis, are clonal disorders of the hematopoietic stem cell in which an uncontrolled proliferation of terminally differentiated myeloid cells occurs. MPNs are characterized by mutations in driver genes, the JAK2V617F point mutation being the most commonly detected genetic alteration in these hematological malignancies. Thus, JAK inhibition has emerged as a potential therapeutic strategy in MPNs, with ruxolitinib being the first JAK inhibitor developed, approved, and prescribed in the management of these blood cancers.
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