Hopp til hovedinnhold

Trombocytose, essensiell

Sist revidert:
Sist revidert av:
Definisjon:
Kronisk myeloproliferativ neoplasi (MPN) med økt trombocyttall >450 x 109 
Forekomst:
Sjelden tilstand, rundt 1 nytt tilfelle per 100 000 innbyggere per år
Symptomer:
Asymptomatisk hos mange, ev. diffuse symptomer som hodepine, svimmelhet, synkope, parestesier, synsforstyrrelser 
Funn:
Mangler ofte. Hos noen kan det foreligge blødninger, trombose, ev. splenomegali
Diagnostikk:
Hb, hvite m/diff, blodutstryk, benmargsbiopsi, mutasjonsanalyser (EDTA bldo) JAK2, CALR, MPL
Diff.diagnoser:
Sekundær trombocytose, trombocytose som ledd i annen MPN inkludert KML
Behandling:
Blodplatehemmende og blodplatesenkende behandling kan være nødvendig
  1. Helsedirektoratet. Nasjonalt handlingsprogram med retningslinjer for diagnostikk, behandling og oppfølging av maligne blodsykdommer. Sist revidert 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. Roaldsnes C, Holst R, Frederiksen H, Ghanima W. Myeloproliferative neoplasms: trends in incidence, prevalence and survival in Norway. Eur J Haematol 2017; 98:85. PubMed  
  4. Forehand CC, Cribb J, May JR. Examination of the relationship between antimicrobials and thrombocytosis. Ann Pharmacother. 2012;46:1425-1429. PubMed  
  5. Tefferi A, Pardanami A. Essential Thrombocythemia. N Engl J Med 2019; 381: 2135-44. pmid:31774958 PubMed  
  6. Tefferi A. Approach to the patient with thrombocytosis. UpToDate, last updated Jan 09, 2019. www.uptodate.com  
  7. Tefferi A et al. Diagnosis and clinical manifestations of essential thrombocythemia. UpToDate, last reviewed Nov 2019. www.uptodate.com  
  8. Mukherjee D. Essential thrombocytosis. BMJ Best Practice, last updated Dec 19, 2014.
  9. Harrison CN, Bareford D, Butt N, et al; British Committee for Standards in Haematology. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol. 2010;149:352-375. PubMed  
  10. Chu DK, Hillis CM, Leong DP, et al. Benefits and Risks of Antithrombotic Therapy in Essential Thrombocythemia: A Systematic Review . Ann Intern Med 2017. doi:10.7326/M17-0284 DOI  
  11. Finazzi G, Ruggeri M, Rodeghiero F, Barbui T. Second malignancies in patients with essential thrombocythaemia treated with busulphan and hydroxyurea: long-term follow-up of a randomized clinical trial. Br J Haematol 2000;110:577-583. PubMed  
  12. Harrison CN, Campbell PJ, Buck G, et al, for the United Kingdom Medical Research Council Primary Thrombocythemia 1 Study. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med 2005; 353: 33-45. New England Journal of Medicine  
  13. Kesler A, Ellis MH, Manor Y, Gadoth N, Lishner M. Neurological complications of essential thrombocytosis (ET). Acta Neurol Scand 2000;102:299-302. PubMed  
  14. Carobbio A, Thiele J, Passamonti F, et al. Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood 2011; : e-pub.
  • Eivind Galteland, overlege dr.med. Avdeling for blodsykdommer, RH, OUS
  • Silje Folven Barlindhaug, spesialist allmennmedisin, redaktør NEL

Tidligere fagmedarbeidere

  • Odd Kildahl-Andersen, overlege, dr. med., Medisinsk avdeling, Universitetssykehuset Nord-Norge, Harstad sykehus
  • Jens Hammerstrøm, overlege, professor dr. med., Hematologisk avdeling, St. Olavs Hospital, Trondheim