Peripheral blood haematopoietic progenitor cells in patients with beta thalassaemia major receiving desferrioxamine or deferiprone as chelation therapy.
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.
Vlachaki
Efthimia
E
Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece.
Ioannidou-Papagiannaki
Elissavet
E
Tziomalos
Konstantinos
K
Haralambidou-Vranitsa
Styliani
S
Perifanis
Vassilios
V
Klonizakis
Ioannis
I
Athanassiou-Metaxa
Miranda
M
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*
D011728:Pyridones; D016179:Granulocyte Colony-Stimulating Factor; D000077543:Deferiprone; D003676:Deferoxamine