Hopp til hovedinnhold

Primær immun trombocytopeni (ITP)

Sist oppdatert: Sist revidert:
Sist revidert av:
Definisjon:
Autoimmun sykdom. Sekundær form kan utløses av infeksjoner, medikamenter og lymfoproliferative sykdommer
Forekomst:
Vanligste årsak til trombocytopeni. Insidens 1,6/100 000 voksne, 5-6/100 000 barn
Symptomer:
Oppdages tilfeldig, ev. på bakgrunn av spontane hudblødninger eller metro-menoragi
Funn:
Normale funn, ev. spontane hudblødninger. OBS! slimhinneblødninger
Diagnostikk:
Virusserologi og ANA kan være aktuelt. Trombocyttantistoff kan påvises hos noen. Beinmargsundersøkelse ved kronisk form eller uklare tilfeller
Behandling:
Akutt form krever sjelden behandling. Immunoglobuliner og glukokortikoider har ofte effekt ved både akutt og kronisk form, kan være forbigående. Splenektomi kan vurderes
  1. Gudbrandsdottir S, Frederiksen H, Birgens HS, et al. Nye behandlingsmuligheder ved primær immun trombocytopeni. Ugeskr Læger 2011; 173: 271. Ugeskrift for Læger  
  2. Pruemer J. Epidemiology, pathophysiology, and initial management of chronic immune thrombocytopenic purpura. Am J Health Syst Pharm 2009; 66 (2 Suppl 2): S4-S10.
  3. Christiansen CF, Bahmanyar S, Ghanima W et al. Chronic immune thrombocytopenia in Denmark, Sweden and Norway: The Nordic Country Patient Registry for Romiplostim. EClinicalMedicine 2019. epub. doi:https://doi.org/10.1016/j.eclinm.2019.07.015
  4. Provan D, Stasi R, Newland AC, et al. International concensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115: 168-86. Blood  
  5. Neunert C, Terrel DR, Arnold DM, et al . American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv 2019; 3: 3829-66. doi:10.1182/bloodadvances.2019000966 DOI  
  6. Schultz NH, Sørvoll IH, Michelsen AE, et al. Thrombosis and Thrombocytopenia after ChAdOx1 nCoV-19 Vaccination. N Engl J Med 2021. pmid:33835768 PubMed  
  7. Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 2009; 113: 2386-93. Blood  
  8. Bach Treppendahl M, Juul Nielsen O. Idiopatisk trombocytopenisk purpura. Ugeskr Læger 2004; 166: 3393-7. PubMed  
  9. Tarantino MD, Bussel JB, Blanchette VS, et al. Romiplostim in children with immune thrombocytopenia: a phase 3, randomised, double-blind, placebo-controlled study. Lancet 2016; 388: 45-54. doi:10.1016/S0140-6736(16)00279-8 DOI  
  10. Grainger JD, Locatelli F, Chgotsampancharoen T, et al. Eltrombopag for children with chronic immune thrombocytopenia (PETIT2): A randomised, multicentre, placebo-controlled trial. Lancet 2015. DOI: 10.1016/S0140-6736(15)61107-2 DOI  
  11. Chugh S, Darvish-Kazem S, Lim W, et al. Rituximab plus standard of care for treatment of primary immune thrombocytopenia: a systematic review and meta-analysis. Lancet Hematology 2015; 2: e71-e85. doi:10.1016/S2352-3026(15)00003-4 DOI  
  12. Iversen PO, Sandset PM. Norsk legemiddelhåndbok, sist endret 07.03.2016. legemiddelhandboka.no  
  13. Buchanan GR, Adix L. Grading of hemorrhage in children with idiopathic thrombocytopenic purpura. J Pediatr 2002; 141: 683-8. pmid:12410198 PubMed  
  14. Wei Y, Ji X, Wang Y, et al. High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood 2016. doi:10.1182/blood-2015-07-659656 DOI  
  15. BlanchetteV, Freedman J, Garvey B. Management of chronic immune thrombocytopenic purpura in children and adults. Semin Hematol 1998; 35: 36-51. PubMed  
  16. Vianelli N, Galli M, de VA, Intermesoli T et al. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica 2005; 90: 72-7. PubMed  
  17. Kojouri K, Vesely SK, Terrell DR, George JN. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications.. Blood 2004; 104: 2623-34. pmid:15217831 PubMed  
  18. Bussel JB, Cheng G, Saleh MN, et al. Eltrombopag for the treatment of chronic idiopathic thrombocytopenic purpura. N Engl J Med 2007; 357: 2237-47. New England Journal of Medicine  
  19. Bussel JB, Provan D, Shamsi T, et al. Effect of eltrombopag on platelets count and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: a randomised, double-blind, placebo-controlled trial. Lancet 2009; 373: 641-8. PubMed  
  20. Wong RSM, Saleh MN, Khelif A et al. Safety and efficacy of long-term treatment of chronic/persistent ITP with eltrombopag: final results of the EXTEND study. Blood 2017; 130: 2527-36. pmid:29042367 PubMed  
  21. Kuiter DJ, Rummel M, Boccia R. Romiplostin or standard of care in patients with immune thrombocytopenia. N Engl J Med 2010; 363: 1959-61. New England Journal of Medicine  
  22. Arnold DM1, Dentali F, Crowther MA et al.. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007; 146: 25-33. pmid:17200219 PubMed  
  23. Waleed Ghanima, Pål André Holme, Geir E. Tjønnfjord. Immune thrombocytopenia--pathophysiology and treatment. Tidsskr Nor Laegeforen 2010; 130: 2120-3. pmid:21052113 PubMed  
  24. Bell WR Jr. Long-term outcome of splenectomy for idiopathic thrombocytopenic purpura. Semin Hematol 2000; 37: 22-5. PubMed  
  25. Bach Treppendahl M, Juul Nielsen O . Kronisk idiopatisk trombocytopenisk purpura. Ugeskr Læger 2004; 166: 3389-93. PubMed  
  26. Portielje JEA, Westendrop RGJ, Kluin-Nelemans HC et al. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001; 97: 2549-54. Blood  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

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