Induction of fetal hemoglobin in the treatment of sickle cell disease

Hematology Am Soc Hematol Educ Program. 2006;58-62. doi: 10.1182/asheducation-2006.1.58.


Reactivation of fetal hemoglobin (HbF) expression is an important therapeutic option in patients with hemoglobin disorders. In sickle cell disease (SCD), an increase in HbF inhibits the polymerization of sickle hemoglobin and the resulting pathophysiology. Hydroxyurea, an inducer of HbF, has already been approved for the treatment of patients with moderate and/or severe SCD. Recent clinical trials with other pharmacological inducers of HbF, such as butyrate and decitabine, have shown considerable promise. In this chapter, we highlight the important clinical trials with pharmacological inducers of HbF, discuss their mechanisms of action and speculate about the future of this therapeutic approach in the treatment of patients with SCD.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Anemia, Sickle Cell / drug therapy*
  • Azacitidine / analogs & derivatives
  • Azacitidine / therapeutic use
  • Butyrates / therapeutic use
  • Decitabine
  • Fetal Hemoglobin / genetics*
  • Gene Expression Regulation / drug effects
  • Humans
  • Hydroxyurea / therapeutic use


  • Butyrates
  • Decitabine
  • Fetal Hemoglobin
  • Azacitidine
  • Hydroxyurea