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Aplastisk anemi

Sist oppdatert: Sist revidert:
Sist revidert av:
Definisjon:
Pancytopeni og hypocellulær beinmarg uten dysplasi. Sannsynligvis autoimmun årsak i majoriteten av tilfellene
Forekomst:
Sjelden. 2-5 tilfeller pr million pr år
Symptomer:
Avhengig av hvilken cellerekke som er mest påvirket. Dyspné, takykardi, residiverende infeksjoner
Funn:
Blekhet, petekkier. Fravær av hepatosplenomegali, lymfadenopati eller benømhet
Diagnostikk:
Hb, hvite m/differensialtelling, trc. Hemolyseparametre. Beinmargsbiopsi bekrefter diagnosen
Behandling:
Avhenger av alder og årsak. Støttende transfusjoner, immunosuppresiv behandling, beinmargstransplantasjon
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  2. Marsh J, Kulasekararaj AG. Aplastic anaemia in adults. BestPractice, last updated August 2018.
  3. Killick SB, Bown N, Cavenagh J, et al; British Society for Standards in Haematology. Guidelines for the diagnosis and management of adult aplastic anaemia. Br J Haematol. 2016;172:187-207. PubMed  
  4. Young NS, Calado RT, Scheinberg P. Current concepts in the pathophysiology and treatment of aplastic anemia. Blood. 2006;108:2509-2519. PubMed  
  5. Iversen PO, Sandset PM. Aplastisk anemi. Norsk legemiddelhåndbok, sist oppdatert 29.02.2016. legemiddelhandboka.no  
  6. Hsu JW, Vogelsang G, Jones RJ and Brodsky RA. Bone marrow transplantation in Shwachman-Diamond syndrome. Bone Marrow Transplant 2002; 30: 255-8. PubMed  
  7. Tischkowitz MD, Hodgson SV. Fanconi anaemia. J Med Genet. 2003;40:1-10. PubMed  
  8. Miano M, Dufour C. The diagnosis and treatment of aplastic anemia: a review. Int J Hematol 2015;101: 527-35. PubMed  
  9. Howard SC, Naidu PE, Hu XJ et al. Natural history of moderate aplastic anemia in children. Pediatr Blood Cancer 2004; 43: 545-51. PubMed  
  10. Frickhofen N, Rosenfeld SJ. Immunosuppressive treatment of aplastic anemia with antithymocyte globulin and cyclosporine. Semin Hematol. 2000;37:56-68. PubMed  
  11. Rosenfeld S, Follmann D, Nunez O and Young NS. Antithymocyte globulin and cyclosporine for severe aplastic anemia: association between hematologic response and long-term outcome. JAMA 2003; 289: 1130-5. Journal of the American Medical Association  
  12. Peffault de Latour R, Kulasekararaj A, Iacobelli S, et al. Eltrombopag Added to Immunosuppression in Severe Aplastic Anemia. N Engl J Med. 2022;386(1):11-23. PubMed  
  13. Brodsky RA, Sensenbrenner LL, Smith BD et al. Durable treatment-free remission following high-dose cyclophosphamide for previously untreated severe aplastic anemia. Ann Intern Med 2001; 135: 477-83. Annals of Internal Medicine  
  14. Jaime-Perez JC, Gonzalez-Llano O and Gomez-Almaguer D. High-dose cyclophosphamide in the treatment of severe aplastic anemia in children. Am J Hematol 2001; 66: 71. PubMed  
  15. Marotta S, Pagliuca S, Risitano AM, Hematopoietic stem cell transplantation for aplastic anemia and paroxysmal nocturnal hemoglobinuria: current evidence and recommendations. Expert Rev Hematol 2014;7: 775-89. PubMed  
  16. Moyo VM, Mukhina GL, Garrett ES and Brodsky RA. Natural history of paroxysmal nocturnal hemoglobinuria using modern diagnostic assays. Br J Haematol 2004; 126: 133-8. PubMed  
  17. Mukhina GL, Buckley JT, Barber JP, Jones RJ and Brodsky RA. Multilineage glycosylphosphatidylinositol anchor deficient hematopoiesis in untreated aplastic anemia. Br J Haematol 2001; 115: 476-82. PubMed  
  18. Camitta BM, Thomas ED, Nathan DG et al. A prospective study of androgens and bone marrow transplantation for treatment of severe aplastic anemia. Blood 1979; 53: 504-14. Blood  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Odd Kildahl-Andersen, overlege, dr. med., Medisinsk avdeling, Universitetssykehuset Nord-Norge, Harstad sykehus
  • Tom Christensen, spesialist i allmennmedisin, Sama legesenter, Harstad
  • Hans Carl Hasselbalch, professor, overlæge, dr.med., Hæmatologisk afd., Roskilde Sygehus, Københavns Universitet