Diagnosis dan Tatalaksana Polisitemia Vera
DOI:
https://doi.org/10.55175/cdk.v47i5.377Kata Kunci:
Eritrosit, JAK2, polisitemiaAbstrak
Polisitemia vera (PV) termasuk kelompok neoplasma mieloproliferatif yang ditandai dengan peningkatan hemoglobin, hematokrit, dan massa sel darah merah. Penyebab utama polisitemia vera adalah mutasi gen pada JAK2, yang mempengaruhi aktivitas sel punca hematopoietik dalam memproduksi eritrosit. Diagnosis berdasarkan kriteria WHO. Terapi bertujuan untuk mencegah komplikasi trombosis dan perdarahan, terdiri dari plebotomi, antiplatelet, dan sitoreduksi. Semua pasien PV membutuhkan plebotomi untuk menjaga tingkat hematokrit, serta aspirin untuk mengurangi risiko trombosis. Obat sitoreduksi hidroksiurea sebagai terapi lini pertama digunakan pada kasus PV berisiko tinggi.
Polycythemia vera is a myeloproliferative neoplasm characterized by an increase in hemoglobin, hematocrit, and red blood cells. The main cause is a gene mutation in JAK2, which affects the activity of producing erythrocytes in hematopoietic stem cells. Diagnosis is based on WHO diagnostic criteria. Therapy aims to prevent complications from thrombosis and bleeding, consisting of phlebotomy, antiplatelet drugs, and cytoreduction. All PV patients need a phlebotomy to maintain hematocrit levels, and aspirin to reduce the risk of thrombosis. Cytoreduction drugs with hydroxyurea as first-line are used high-risk PV.
Unduhan
Referensi
Tefferi A, Vannucchi AM, Barbui T. Polycythemia vera treatment algorithm 2018. Blood Cancer J. 2018;8:3
Ibrahim IK, Hassan R, Ali EW, Omer A. Polycythaemia vera among Sudanese patients with special emphasis on JAK2 mutations. Asian Pac J Cancer Prev. 2019;20(1):41-4
Zivot A, Lipton JM, Narla A, Blanc L. Erythropoiesis: Insights into pathophysiology and treatments in 2017. Molecular Med. 2018;24(1):11
Maffioli M, Mora B, Passamonti F. Polycythemia vera: From New, Modified Diagnostic Criteria to New Therapeutic Approaches. Clinical Advances in Hematology & Oncology. 2017;15(9):700-7
Greenfield G, McPherson S, Mills K, McMullin MF. The ruxolitinib effect: Understanding how molecular pathogenesis and epigenetic dysregulation impact therapeutic efficacy in myeloproliferative neoplasms. Journal of Translational Medicine. 2018;16:360
Milosevic JD, Kralovics R. Genetic and epigenetic alterations of myeloproliferative disorders. Int J Hematol. 2013;97:183-97
U.S National Library of Medicine. Genetics Home Reference [Internet]. 2019 [cited 2019 March 27. Available from: https://ghr.nlm.nih.gov/gene/JAK2
Helbig G. Classical Philadelphia-negative myeloproliferative neoplasms: Focus on mutations and JAK2 inhibitors. Medical Oncology. 2018;35(9):119
de Freitas RM, Maranduba CMdC. Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview. Revista Brasileira de Hematologia e Hemoterapia. 2015;37(5):348-53.
McMullin MF, Wilkins BS, Harrison CN. Management of polycythemia vera: A critical review of current data. British Journal of Hematology. 2015;172(3):337-49
Passamonti F, Rumi E, Caramella M, Elena C, Arcaini L, Boveri E, et al. A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis. Blood 2008; 111(7):3383-7
Klein-Weigel PF, Volz TS, Richter JG. Erythromelalgia. Vasa. 2018; 47(2):91-7.
Siegel FP, Tauscher J, Petrides PE. Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 pateints. Am J Hematol. 2013;88(8):665-9
Devendra K, Falchi L, Verstovsek S. The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: A review. Ann Hematol. 2017; 96(10):1595-604
Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am J Hematol. 2019; 94:133-44
Vannucchi AM. How I treat polycythemia vera. Blood. 2014;124:3212-20
Scott LM, Tong W, Levine R, Scott MA, Beer PA, Stratton MR, et al. JAK2 exon mutations in polycythemia vera and idiopathic erythrocytosis. New England Journal Medicine. 2007;365(5):459-68
Singh A, Xu YJ. The cell killing mechanisms of hydroxyurea. Genes. 2016;7(11):99
Hasselbalch HC, Holmström MO. Perspectives on interferon-alpha in the treatment of polycythemia vera and related myeloproliferative neoplasms: Minimal residual disease and cure? Seminars in Immunopathology. 2019;41(1):5-19
Larran AA, Aviles LM, Boluda JC, Marin FF, Antelo ML, Burgaleta C. Busulfan in pateints with polycythemia vera or essential thrombocythemia refractory or intolerant to hydroxyurea. Annals of Hematology. 2014;93(12):2037-43.
Begna K, Abdelatif A, Schwager S, Hanson C, Pardanani A, Tefferi A. Busulfan for the treatment of myeloproliferative neoplasms: the Mayo Clinic experience. Blood Cancer Journal. 2016;6(5):427.
Raedler LA. Jakafi (ruxolitinib): First FDA-approved medication for the treatment of patients with polycythemia vera. Am Health Drug Benefits. 2015;8:75-9
Bryan J, Verstovsek S. Overcoming treatment challenges in myelofibrosis and polycythemia vera: The role of ruxolitinib. Cancer Chemother Pharmacol. 2016;77(6):1125-42
Klemencic S, Perkins J. Diagnosis and management of oncologic emergencies. West J Emerg Med. 2019;20(2):316–22.
Ma L, Chen Y, Jin G, Yang Y, Ga Q, Ge RL. Vascular endothelial growth factor as a prognostic parameter in subjects with "Plateau Red Face". High Altitude Med Biol. 2015;16(2):147–53. doi:10.1089/ham.2014.1091
Unduhan
Diterbitkan
Cara Mengutip
Terbitan
Bagian
Lisensi
Hak Cipta (c) 2020 Cermin Dunia Kedokteran
Artikel ini berlisensi Creative Commons Attribution-NonCommercial 4.0 International License.
