Introduction: Polycythemia vera is a myeloproliferative neoplasm (MPN) characterized by increased red blood cell mass. The natural evolution of this MPN is to progress to an anemic/cytopenic phase also known as "spent" phase prior to transformation into an accelerated and/or an overt leukemic phase.
Case Report: Herein, we describe a case of a patient with polycythemia vera transitioning though a "spent" phase to an MPN in accelerated phase (MPN-AP). The patient had anemia, thrombocytopenia, neutrophilia and increased blasts in the bone marrow. Upon treatment with four cycles of 5-azacitidine, the patient's polycythemia vera reversed back to the proliferative phase. Serial phlebotomies were again required.
Discussion: Reversal of a "spent" phase by 5-azacitidine back to a proliferative polycythemia vera phase requiring phlebotomies has not been previously reported in the scientific literature. We might witness similar cases in the literature in the future years, which could lead to yet another therapeutic indication of this important pharmacologic agent.
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http://dx.doi.org/10.1177/1078155220901769 | DOI Listing |
Intern 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.
Br J Haematol
January 2025
The First Affiliated Hospital, Zhejiang University School of Medicine, Zhejiang, Hangzhou, China.
Children (Basel)
December 2024
Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan.
Introduction: Polycythemia is a rare condition that can be either primary or secondary. We report a case of an adolescent with progressive hydronephrosis-induced polycythemia and low erythropoietin levels, along with a thorough literature review.
Report Of A Case: A 17-year-old girl with epilepsy had progressively elevated hemoglobin levels and low erythropoietin levels.
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