BMSEHA15
Published online 8 October 2009
Haematologica, Vol 95, Issue 3, 398-405 doi:10.3324/haematol.2009.012773
Copyright © 2010 by Ferrata Storti Foundation
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Myelodysplastic Syndromes

Unimpaired terminal erythroid differentiation and preserved enucleation capacity in myelodysplastic 5q(del) clones: a single cell study

Laurent Garderet1,2,3, Ladan Kobari1,2, Christelle Mazurier1,4, Caroline De Witte1,2, Marie-Catherine Giarratana1,2, Christine Pérot5, Norbert Claude Gorin1,2,3, Hélène Lapillonne1,2,6, Luc Douay1,2,4,6

1 INSERM, UMR_S 938, Paris;
2 UPMC Univ Paris 06, UMR_S 938, Paris;
3 AP-HP, Hôpital Saint Antoine, Service d’Hématologie et de Thérapie Cellulaire, Paris;
4 Etablissement Français du Sang Ile de France, Ivry-sur-Seine;
5 AP-HP, Hôpital Saint Antoine, Service de Cytogénétique, Paris and
6 AP-HP, Hôpital Armand Trousseau, Service d’Hématologie Biologique, Paris, France

Correspondence: Laurent Garderet, 184, rue du Faubourg Saint Antoine, Paris 75012, France., E-mail: laurent.garderet{at}sat.aphp.fr

Background: Anemia is a characteristic of myelodysplastic syndromes, such as the rare 5q- syndrome, but its mechanism remains unclear. In particular, data are lacking on the terminal phase of differentiation of erythroid cells (enucleation) in myelodysplastic syndromes.

Design and Methods: We used a previously published culture model to generate mature red blood cells in vitro from human hematopoietic progenitor cells in order to study the pathophysiology of the 5q- syndrome. Our model enables analysis of cell proliferation and differentiation at a single cell level and determination of the enucleation capacity of erythroid precursors.

Results: The erythroid commitment of 5q(del) clones was not altered and their terminal differentiation capacity was preserved since they achieved final erythroid maturation (enucleation stage). The drop in red blood cell production was secondary to the decrease in the erythroid progenitor cell pool and to impaired proliferative capacity. RPS14 gene haploinsufficiency was related to defective erythroid proliferation but not to differentiation capacity.

Conclusions: The 5q- syndrome should be considered a quantitative rather than qualitative bone marrow defect. This observation might open the way to new therapeutic concepts.

Key words: myelodysplastic syndrome, 5q- syndrome, erythroid differentiation, enucleation.


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M. Jadersten
Pathophysiology and treatment of the myelodysplastic syndrome with isolated 5q deletion
Haematologica, March 1, 2010; 95(3): 348 - 351.
[Full Text] [PDF]