title: Peripheral blood haematopoietic progenitor cells in patients with beta thalassaemia major receiving desferrioxamine or deferiprone as chelation therapy.
abstract: OBJECTIVE
  The main adverse effect of deferiprone is the development of neutropenia, which occurs via an unknown mechanism. We aimed to gain insight into the pathogenesis of deferiprone-induced neutropenia by assessing the peripheral blood haematopoietic progenitor cells.
  
  
  METHODS
  Sixteen patients with beta thalassaemia were studied; nine (Group A) were receiving desferrioxamine and seven (Group B) deferiprone. Ten healthy individuals comprised the control group (Group C).
  
  
  RESULTS
  Granulocyte-erythrocyte-monocyte-megakaryocyte colony forming units were significantly more in Groups A and B compared with Group C. Granulocyte-macrophage colony forming units (CFU-GM) were significantly more in Group B compared with Group C. Macrophage colony forming units were significantly less in Group B compared with Group C. Granulocyte colony forming units (CFU-G) were significantly more in Group A compared with Group C. We found a trend in the difference in the number of CFU-G between patients' groups (P = 0.123). Adding serum from patients receiving deferiprone to cultures of controls resulted in a maturation arrest of the granulocytic lineage.
  
  
  CONCLUSIONS
  Our findings point to a maturation arrest at the level of CFU-GM as a potential mechanism of deferiprone-induced neutropenia.
authors.affiliation: Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece.
authors.forename: Efthimia
authors.initials: E
authors.lastname: Vlachaki
authors.forename: Elissavet
authors.initials: E
authors.lastname: Ioannidou-Papagiannaki
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Tziomalos
authors.forename: Styliani
authors.initials: S
authors.lastname: Haralambidou-Vranitsa
authors.forename: Vassilios
authors.initials: V
authors.lastname: Perifanis
authors.forename: Ioannis
authors.initials: I
authors.lastname: Klonizakis
authors.forename: Miranda
authors.initials: M
authors.lastname: Athanassiou-Metaxa
mesh_terms: D000328:Adult; D015913:Chelation Therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000494:pharmacology* / Q000627:therapeutic use*; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000187:drug effects; D006412:Hematopoietic Stem Cells / Q000187:drug effects*; D006801:Humans; D008297:Male; D009503:Neutropenia / Q000139:chemically induced; D011728:Pyridones / Q000009:adverse effects / Q000494:pharmacology* / Q000627:therapeutic use*; D012016:Reference Values; D016896:Treatment Outcome; D017086:beta-Thalassemia / Q000188:drug therapy*
chemical_list: D011728:Pyridones; D016179:Granulocyte Colony-Stimulating Factor; D000077543:Deferiprone; D003676:Deferoxamine