Pathofysiologie en behandeling van sikkelcelziekte

Translated title of the contribution: Pathophysiology and treatment of sickle-cell disease

E J van Beers, M Peters, B J Biemond

Research output: Contribution to journalArticleAcademicpeer-review


Sickle-cell disease is a hereditary haemoglobinopathy caused by a mutation in the beta-globin gene. The disease is characterised by recurrent vaso-occlusive crises resulting in severe organ damage and a sharply reduced life expectancy. The formation of haemoglobin-S polymers in hypoxic conditions plays a pivotal role in sickle-cell disease and produces the characteristic phenotype of sickle-shaped erythrocytes that promote vasoocclusion. Endothelial cell activation, enhanced erythrocyte and leukocyte adhesion, vasoconstriction and coagulation activation play an important role in vaso-occlusive crises. Treatment of pain and hydration remain the main interventions in the management ofvaso-occlusive crises. Hydroxyurea has been shown to prevent vaso-occlusive crises by increasing the amount of foetal haemoglobin. Allogeneic stem-cell transplantation is the only curative therapy. However, transplantation-related mortality, graft-versus-host disease and the limited availability of HLA-identical donors restrict this therapeutic option.

Translated title of the contributionPathophysiology and treatment of sickle-cell disease
Original languageDutch
Pages (from-to)1144-9
Number of pages6
JournalNederlands Tijdschrift voor Geneeskunde
Issue number21
Publication statusPublished - 2005
Externally publishedYes


  • Anemia, Sickle Cell
  • Antisickling Agents
  • Cell Adhesion
  • Combined Modality Therapy
  • Erythrocytes
  • Humans
  • Hydroxyurea
  • Stem Cell Transplantation


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