Background: Polycythaemia vera (PV) is a Philadelphia-negative myeloproliferative neoplasm, typically driven by acquired JAK2 mutation and characterised by elevated red cell mass and increased risk of thrombotic events. Patients are managed with phlebotomy to maintain haematocrit (Hct) < 0.45, and patients stratified as 'high risk' for thrombosis are additionally treated with cytoreductive agents to attain this target.
Study: This analysis of newly diagnosed JAK2 mutant PV patients (n = 50) over 2 years aimed to determine how effectively patients attained and maintained target Hct according to recommended practice.
Conclusions: We found that patients spent the majority of time in target Hct range. Findings are supportive of current management guidelines.
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http://dx.doi.org/10.1007/s11845-023-03510-7 | DOI Listing |
Arch Med Res
January 2025
Cancer Molecular Diagnostics, St. James's Hospital, Dublin, Ireland. Electronic address:
Ann Hematol
January 2025
Hematology and Hematopoietic Stem Cell Transplant Center, Department of Medicine and Surgery, University of Naples Federico II, Via S. Pansini 5, Naples, 80131, Italy.
Splenomegaly is a quite common clinical feature of Philadelphia (Ph) negative chronic myeloproliferative neoplasms (MPNs) and its presence may, in some cases, drives treatment decision. Most importantly, palpable splenomegaly is a minor criterion for both pre-fibrotic/early primary myelofibrosis and primary myelofibrosis (PMF) diagnosis, even if clinical assessment by physical examination is poorly reliable and accurate. On the other hand, despite the International Working Group-Myeloproliferative Neoplasms Research and Treatment and European LeukemiaNet guidelines defined spleen response criteria by palpation, they also recognized the highly subjective nature of spleen size assessment by physical examination, and recommended objective confirmation of volume reduction via computed tomography or magnetic resonance imaging (MRI).
View Article and Find Full Text PDFIntern Med
January 2025
Department of Internal Medicine 1, Shimane University Faculty of Medicine, Japan.
We herein report a 56-year-old man with severe hypocalcemia during ruxolitinib therapy for myelofibrosis transitioning from JAK2 mutation-positive polycythemia vera. Blood transfusions were administered every one to two weeks for ruxolitinib-induced anemia. Blood tests revealed hypocalcemia with low TRACP-5b, 25-hydroxyvitamin D (25 (OH) D), and 1,25-dihydroxyvitamin D (1,25 (OH) D) levels within the lower reference range.
View Article and Find Full Text PDFAnn Hematol
January 2025
Department of Medicine, School of Clinical Medicine, LKS Faculty of Medicine, the University of Hong Kong, Hong Kong, China.
Polycythemia vera (PV) is characterized by clonal hematopoietic stem or progenitor cells with constitutively active somatic mutation(s) in the Janus kinase 2 gene. Phlebotomy (Phl) and aspirin are often used alone for low-risk PV patients. However, data from the Low-PV study demonstrated that Phl and aspirin may not be adequate for patients.
View Article and Find Full Text PDFBr J Haematol
January 2025
CRIMM, Azienda Ospedaliera Universitaria Careggi, Dipartimento di Medicina Sperimentale e Clinica, Università di Firenze, Florence, Italy.
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