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Aims: Spleen and liver stiffness, investigated by VCTE (Vibration-Controlled Transient Elastography), have been associated with marrow fibrosis in patients with myeloproliferative neoplasms (MPNs). Tissue stiffness can be assessed by shear wave point (pSWE) and bidimensional elastography (2DSWE). Spleen stiffness (SS) values were higher in Myelofibrosis (MF) and Polycythemia Vera (PV) compared to Essential Thrombocythemia (ET). We aimed to identify SWE differences between MPN patients and healthy volunteers; to evaluate specific SWE features in patients with MF, PV and ET; to establish a correlation with bone marrow fibrosis in patients with myeloproliferative disease.
Methods: Patients with myeloproliferative disease and healthy volunteers performed evaluation of spleen and liver stiffness (LS) by pSWE and 2DSWE.
Results: A total of 218 subjects were included: 143 with myeloproliferative disease (64 MF, 29.4%, 33 PV, 15.1% and 46 ET, 21.1%), and 75 (34.4%) healthy volunteers. Compared to volunteers, MF patients had greater spleen (pSWE 40.9 vs. 26.3 kPa, p < 0.001; 2DSWE 34.9 vs. 20.1 kPa, p < 0.001), and liver stiffness (pSWE 7.72 vs. 5.52 kPa, p < 0.001; 2DSWE 6.96 vs. 5.01 kPa, p < 0.001). In low (0-1) (n = 81, 60.4%) versus high-grade bone marrow fibrosis (2-3) (n = 42, 39.6%), is evident a higher median stiffness in patients with higher grades of fibrosis both for liver (pSWE 5.2 vs. 6.65 kPa; 2DSWE 5.1 vs. 6.05 kPa) and spleen (pSWE 27.2 vs. 37.9 kPa, 2DSWE 21.7 vs 30.75 kPa-p < 0.001 in both).
Conclusion: SWE evaluation distinguishes MF patients from HV and ET/PV and may help in MPN diagnosis. LS and SS values are associated with bone marrow fibrosis grade.
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http://dx.doi.org/10.1007/s40477-024-00932-6 | DOI Listing |
Int J Hematol
September 2025
MLL Munich Leukemia Laboratory, Max-Lebsche-Platz 31, 81377, Munich, Germany.
Chronic myeloid leukemia (CML) and BCR::ABL1-negative MPN were thought to be mutually exclusive, but synchronous and sequential cases have been reported. We screened 35,001 patients for BCR::ABL1 fusion or JAK2, CALR, or MPL mutations to investigate the sequential development of CML and BCR::ABL1 negative-MPNs. We discovered that 5.
View Article and Find Full Text PDFJ Hematol
August 2025
Department of Oncology, Edwards Comprehensive Cancer Institute, Marshall University, Huntington, WV 25701, USA.
Background: Myelofibrosis (MF) can be primary (PMF) or secondary (SMF), with PMF driven by Janus kinases-signal transducer and activator of transcription proteins (JAK-STAT) pathway activation due to Janus kinase 2 (), the thrombopoietin receptor gene (myeloproliferative leukemia virus oncogene ()), or calreticulin () mutations. Nearly 50% of PMF patients experience anemia (hemoglobin (Hb) < 10 g/dL), often worsened by JAK inhibitors like ruxolitinib and fedratinib. Momelotinib, an oral ACVR1, JAK1, and JAK2 inhibitor, improves anemia, symptoms, and splenomegaly, likely through hepcidin regulation.
View Article and Find Full Text PDFAnn Med Surg (Lond)
September 2025
University of Debrecen, Debrecen, Hungary.
Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells characterized by aberrant proliferation of myeloid lineages, driven primarily by mutations in JAK2, CALR, and myeloproliferative leukemia, leading to constitutive activation of the JAK-STAT pathway. Emerging evidence highlights mitochondrial dysfunction as a key factor in MPN pathogenesis, contributing to increased reactive oxygen species production, mitochondrial DNA mutations, and dysregulated mitochondrial dynamics, which collectively promote clonal expansion and apoptosis resistance. Targeting mitochondrial pathways has gained attention as a therapeutic strategy, with approaches including mitochondria-targeted antioxidants, metabolic inhibitors, and modulation of mitophagy and mitochondrial fission/fusion dynamics.
View Article and Find Full Text PDFAm J Hematol
September 2025
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
Survival prediction models in essential thrombocythemia (ET) include the International Prognostic Scoring System (IPSET) and the more recently introduced triple-A (AAA) prognostic score. The latter enlists age and absolute neutrophil (ANC) and lymphocyte (ALC) counts as risk variables. In the current study, a Mayo Clinic discovery cohort of 658 patients with ET was used to identify AAA-independent risk variables.
View Article and Find Full Text PDFMedicine (Baltimore)
August 2025
Department of Neurology, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Rationale: Cerebral venous and sinus thrombosis (CVST) is a rare and special type of cerebrovascular disease characterized by complex etiology, diverse forms of onset, nonspecific clinical manifestations, and difficult diagnosis. Myeloproliferative neoplasm-unclassifiable (MPN-U) refers to clinical manifestations, laboratory findings, and morphological features that are consistent with the diagnosis of myeloproliferative neoplasms, but do not meet further specific classification criteria, or have overlapping features of 2 or more myeloproliferative neoplasms. Here, we report a case of CVST with MPN-U and a history of head trauma.
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