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Myelodysplastiske syndromer

Sist revidert:
Sist revidert av:


Definisjon:
Malign stamcellesykdom i beinmarg med dysplastisk og ineffektiv produksjon av blodceller med perifer cytopeni (anemi og/eller trombocytopeni og/eller granulocytopeni) 
Forekomst:
Insidens om lag 4-5/100 000 innbyggere per år, økende med alder
Symptomer:
Ofte vage symptomer tidlig, men etterhvert slapphet (anemi), hudblødninger, og økt infeksjonstendens
Funn:
Varierende funn, blekhet og hudblødninger vanligst
Diagnostikk:
Blodprøver: anemi med lave retikulocytter, eventuelt nøytropeni og/eller trombocytopeni. Beinmargsutstryk gir normalt sett diagnosen, men klassifikasjon kan være vanskelig
Behandling:
Cellegift, ev. allogen stamcelletransplantasjon i egnede tilfeller. Allo-SCT er eneste kurative mulighet
  1. Nasjonalt handlingsprogram med retningslinjer for diagnostikk, behandling og oppfølging av maligne blodsykdommer. Myelodysplastiske syndromer. Helsedirektoratet 27.05.2020. www.helsebiblioteket.no  
  2. Nordic MDS Group. Guidelines for the diagnosis and treatment of Myelodysplastic Syndrome and Chronic Myelomonocytic Leukemia. 8th update, May 2017 www.nmds.org  
  3. Steensma DP. Myelodysplastic syndromes current treatment algorithm 2018. Blood Cancer J. 2018;8(5):47. Published 2018 May 24. PMID: 29795386 PubMed  
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  7. Molldrem JJ, Leifer E, Bahceci E et al. Antithymocyte globulin for treatment of the bone marrow failure asssociated with myelodysplastic syndromes. Ann Intern Med 2002;137:156-63. Annals of Internal Medicine  
  8. Musto P, Maurillo L, Spagnoli A, et al. Azacitidine for the treatment of lower risk myelodysplastic syndromes : a retrospective study of 74 patients enrolled in an Italian named patient program. Cancer 2010; 116: 1485. PubMed  
  9. van der Helm LH, Alhan C, Wijermans PW, et al. Platelet doubling after the first azacitidine cycle is a promising predictor for response in myelodysplastic syndromes (MDS), chronic myelomonocytic leukaemia (CMML) and acute myeloid leukaemia (AML) patients in the Dutch azacitidine compassionate named patient programme. Br J Haematol 2011; 155: 599-606. PubMed  
  10. Sauntharajah Y, Nakamura R, Nam J et al. HLA-DR15(DR2) is overrepresented in myelodysplastic syndrome and aplastic anemia and predicts a response to immunosuppression in myelodysplastic syndrome. Blood 2002; 100:1570-4. PubMed  
  11. Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997; 89: 2079. Blood  
  12. Nordic MDS Group. Guidelines for the diagnosis and treatment of Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia. 5 utg. Stockholm: Nordic MDS Group; 2011. www.nmds.org  
  13. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H et al., red. WHO classification of tumours of haematopoietic and lymphoid tissue. 4 utg. Lyon: IARC; 2008.
  • Ingard Løge, spesialist allmennmedisin, redaktør NEL

Tidligere fagmedarbeidere

  • Odd Kildahl-Andersen, overlege, dr. med., Medisinsk avdeling, Universitetssykehuset Nord-Norge, Harstad sykehus
  • Tom Christensen, spesialist i allmennmedisin, Sama legesenter, Harstad