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Polycytemia vera (PV)

Sist revidert:
Sist revidert av:
Definisjon:
PV er polycytemi forårsaket av en malign myeloproliferativ sykdom
Forekomst:
Årlig insidens i Norge er mellom 2-3/100 000
Symptomer:
Er mangfoldige og diffuse, mest typisk er kløe etter varme bad, slapphet - noen er asymptomatiske
Funn:
Mest typiske er rødmusset ansikt - pletorisk utseende, splenomegali, ev. hepatomegali
Diagnostikk:
Tilleggsundersøkelser er Hb, Htc, trombocytter, hvite m/diff, erytropoietin, JAK2 mutasjonsanalyse
Behandling:
Hovedbehandlingen er venesectio, ASA og ev. cytoreduktiv behandling
  • Håvar Knutsen, pensjonert hematolog, Hematologisk avdeling, OUS Rikshospitalet, Oslo
  1. Helsedirektoratet. Nasjonalt handlingsprogram med retningslinjer for diagnostikk, behandling og oppfølging av maligne blodsykdommer. Myeloproliferative neoplasier. 2012. Sist faglig oppdatert 23. des 2021. www.helsedirektoratet.no  
  2. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 2020 Dec;95(12):1599-1613. PMID: 32974939.
  3. Vainchenker W, Kralovics R. Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms. Blood. 2017 Feb 9;129(6):667-679. PMID: 28028029.
  4. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.Blood 2016; 127(20): 2391-405. Blood  
  5. Busque L, Porwit A, Day R, et al. Laboratory Investigation of Myeloproliferative Neoplasms. AJCP Am J Clin Pathol 2016; 146: 408-422. PubMed  
  6. Tefferi A, Guglielmelli P, Lasho TL et al. Mutation-enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera. Br J Haematol. 2020 Apr;189(2):291-302. PMID: 31945802.
  7. Stein BL, Oh ST, Berenzon D, et al. Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F. J Clin Oncol 2015 ;33(33): 3953-60. DOI: 10.1038/s41375-018-0207-9 DOI  
  8. McMullin MF, Wilkins BS, Harrison CN. Management of polycythaemia vera: a critical review of current data. Br J Haematol 2016; 172(3): 337-49. PubMed  
  9. Harrison CN, Robinson SE. Myeloproliferative disorders in pregnancy. Hematol Oncol Clin North Am 2011; 25(2): 261-75. PubMed  
  10. Griesshammer M, Kiladjian JJ, Besses C. Thromboembolic events in polycythemia vera. Ann Hematol. 2019;98(5):1071-1082. PubMed  
  11. Gisslinger H, Zagrijtschuk O, Buxhofer-Ausch V, et al. Ropeginterferon alfa-2b, a novel IFNa-2b, induces high response rates with low toxicity in patients with polycythemia vera. Blood. 2015; 126(15): 1762-1769. PubMed  
  12. Gowin K, Thapaliya P, Samuelson J, et al. Pegylated interferon alpha - 2a is clinically effective and tolerable in myeloproliferative neoplasm patients treated off clinical trial. Leuk Res. 2017; 54: 73-77. PubMed  
  13. Vannucchi AM, Kiladjian JJ, Griesshammer M, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015; 372(5): 426-35. PubMed  
  14. Passamonti F, Griesshammer M, Palandri F, et al. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study. Lancet Oncol 2017; 18: 88-99. PubMed  
  15. Ahlstrand E, Andersen CL, Andreasson B et al. Nordic care program for patients with Essential Thrombocythemia, Polycythemia Vera. 4th version March 2017. Stockholm: Nordic MPD Study Group; 2017. and Primary Myelofibrosis legehandboka.no  
  16. Barbui T, Barosi G, Birgegard G, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol 2011; 29(6): 761-70. PubMed  
  17. Szuber N, Vallapureddy RR, Penna D, et al. Myeloproliferative neoplasms in the young: Mayo Clinic experience with 361 patients age 40 years or younger. Am J Hematol. 2018; 93: 1474–1484.
  • Eivind Galteland, overlege dr.med. Avdeling for blodsykdommer, RH, OUS
  • Silje Folven Barlindhaug, spesialist i allmennmedisin, redaktør NEL