From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review
Introduction and purpose: Polycythemia vera (PV) is a chronic myeloproliferative neoplasm, part of the group of Philadelphia-negative neoplasms alongside essential thrombocythemia and myelofibrosis. It is characterized by elevated red blood cell mass, frequently accompanied by leukocytosis and thro...
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Kazimierz Wielki University
2025-07-01
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| Series: | Journal of Education, Health and Sport |
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| Online Access: | https://apcz.umk.pl/JEHS/article/view/61765 |
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| author | Katarzyna Siekaniec Bartosz Roś Natalia Kuchenbeker Adriana Dojs Julia Mierzwińska-Mucha Magdalena Jakubowicz Wojciech Kowalewski |
| author_facet | Katarzyna Siekaniec Bartosz Roś Natalia Kuchenbeker Adriana Dojs Julia Mierzwińska-Mucha Magdalena Jakubowicz Wojciech Kowalewski |
| author_sort | Katarzyna Siekaniec |
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Introduction and purpose: Polycythemia vera (PV) is a chronic myeloproliferative neoplasm, part of the group of Philadelphia-negative neoplasms alongside essential thrombocythemia and myelofibrosis. It is characterized by elevated red blood cell mass, frequently accompanied by leukocytosis and thrombocytosis. A JAK2 mutation is present in about 95% of cases, typically with low erythropoietin levels. This study aims to review the current understanding of PV, including its pathophysiology, diagnostic criteria, treatment options, and emerging therapies.
A brief description of the state of knowledge: PV is rare, with an incidence of 0.01–4 cases per 100,000 annually, and is usually diagnosed between the ages of 60 and 65. Symptoms stem from increased blood viscosity and include headaches, aquagenic pruritus, thrombosis, and splenomegaly. Diagnosis relies on elevated hemoglobin/hematocrit, bone marrow findings, and JAK2 mutation. According to ICC and WHO criteria, diagnosis can sometimes be made without bone marrow biopsy. Standard treatment includes low-dose aspirin and phlebotomy, with cytoreductive therapy (hydroxyurea or pegylated interferon) based on thrombotic risk. New drugs such as rusfertide, idasanutlin, and givinostat show promise in symptom control and potential disease modification.
Summary: While current therapies effectively manage hematocrit and thrombotic risk, they do not address the root cause of PV. Novel treatments targeting molecular mechanisms may improve quality of life and reduce the risk of progression to myelofibrosis or acute leukemia. Further research is needed to develop curative strategies.
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| format | Article |
| id | doaj-art-d6e5d6efef914e0683f23fec4f7f3d0d |
| institution | Matheson Library |
| issn | 2391-8306 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Kazimierz Wielki University |
| record_format | Article |
| series | Journal of Education, Health and Sport |
| spelling | doaj-art-d6e5d6efef914e0683f23fec4f7f3d0d2025-07-06T07:20:39ZengKazimierz Wielki UniversityJournal of Education, Health and Sport2391-83062025-07-018310.12775/JEHS.2025.83.61765From Diagnosis to Health Practice: Polycythemia Vera. A Literature ReviewKatarzyna Siekaniec0https://orcid.org/0009-0000-3454-4987Bartosz Roś1https://orcid.org/0009-0003-9827-1385Natalia Kuchenbeker2https://orcid.org/0009-0005-0437-7424Adriana Dojs3https://orcid.org/0009-0003-7411-7172Julia Mierzwińska-Mucha4https://orcid.org/0009-0003-2829-1815Magdalena Jakubowicz5https://orcid.org/0009-0009-0442-7507Wojciech Kowalewski6https://orcid.org/0009-0001-0696-3137Division of Medical Humanities and Social Science, Department of Humanities and Social Science, Wroclaw Medical University, ul. Mikulicza-Radeckiego 7, 50-368 Wroclaw, PolandDivision of Anatomy, Department of Human Morphology and Embryology, Faculty of Medicine, Wroclaw Medical University, ul. Chałubińskiego 6a, 50-368 Wroclaw, Poland Faculty of Medicine, Wroclaw Medical University, ul. Wyb. L. Pasteura 1, 50-367 Wroclaw, PolandFaculty of Medicine, Wroclaw Medical University, ul. Wyb. L. Pasteura 1, 50-367 Wroclaw, Poland Pod Paprocią Pharmacy, Rynek 8, 57-100 Strzelin, Poland4 Military Clinical Hospital With Polyclinic SPZOZ, ul. R. Weigla 5, 50-981 Wroclaw, Poland Wrocław University Hospital Borowska 213, 50-556 Wrocław, Poland Introduction and purpose: Polycythemia vera (PV) is a chronic myeloproliferative neoplasm, part of the group of Philadelphia-negative neoplasms alongside essential thrombocythemia and myelofibrosis. It is characterized by elevated red blood cell mass, frequently accompanied by leukocytosis and thrombocytosis. A JAK2 mutation is present in about 95% of cases, typically with low erythropoietin levels. This study aims to review the current understanding of PV, including its pathophysiology, diagnostic criteria, treatment options, and emerging therapies. A brief description of the state of knowledge: PV is rare, with an incidence of 0.01–4 cases per 100,000 annually, and is usually diagnosed between the ages of 60 and 65. Symptoms stem from increased blood viscosity and include headaches, aquagenic pruritus, thrombosis, and splenomegaly. Diagnosis relies on elevated hemoglobin/hematocrit, bone marrow findings, and JAK2 mutation. According to ICC and WHO criteria, diagnosis can sometimes be made without bone marrow biopsy. Standard treatment includes low-dose aspirin and phlebotomy, with cytoreductive therapy (hydroxyurea or pegylated interferon) based on thrombotic risk. New drugs such as rusfertide, idasanutlin, and givinostat show promise in symptom control and potential disease modification. Summary: While current therapies effectively manage hematocrit and thrombotic risk, they do not address the root cause of PV. Novel treatments targeting molecular mechanisms may improve quality of life and reduce the risk of progression to myelofibrosis or acute leukemia. Further research is needed to develop curative strategies. https://apcz.umk.pl/JEHS/article/view/61765polycythemia veraJAK2phlebothomy |
| spellingShingle | Katarzyna Siekaniec Bartosz Roś Natalia Kuchenbeker Adriana Dojs Julia Mierzwińska-Mucha Magdalena Jakubowicz Wojciech Kowalewski From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review Journal of Education, Health and Sport polycythemia vera JAK2 phlebothomy |
| title | From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review |
| title_full | From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review |
| title_fullStr | From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review |
| title_full_unstemmed | From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review |
| title_short | From Diagnosis to Health Practice: Polycythemia Vera. A Literature Review |
| title_sort | from diagnosis to health practice polycythemia vera a literature review |
| topic | polycythemia vera JAK2 phlebothomy |
| url | https://apcz.umk.pl/JEHS/article/view/61765 |
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