TY - JOUR
T1 - Changes in RPS14 expression levels during lenalidomide treatment in Low- and Intermediate-1-risk myelodysplastic syndromes with chromosome 5q deletion
AU - Oliva, Esther N.
AU - Cuzzola, Maria
AU - Nobile, Francesco
AU - Ronco, Francesca
AU - D'Errigo, Maria Grazia
AU - Laganà, Carmelo
AU - Morabito, Fortunato
AU - Galimberti, Sara
AU - Cortelezzi, Agostino
AU - Aloe Spiriti, Maria A.
AU - Specchia, Giorgina
AU - Poloni, Antonella
AU - Breccia, Massimo
AU - Ghio, Riccardo
AU - Finelli, Carlo
AU - Iacopino, Pasquale
AU - Alimena, Giuliana
AU - Latagliata, Roberto
PY - 2010/9
Y1 - 2010/9
N2 - Background: Haploinsufficiency of the ribosomal protein S14 RPS14 gene, located in the common deleted region of chromosome 5q, is a potential causal factor of 5q- syndrome. Lenalidomide elicits high response rates and morphological improvements in myelodysplastic syndrome (MDS) patients with chromosome 5q deletion [del(5q)]. Methods: To further evaluate the role of RPS14, its transcription was tested in bone marrow cells from 17 patients with International Prognostic Scoring System defined Low- or Intermediate-1-risk MDS with del(5q) as a single or additional cytogenetic abnormality receiving treatment with lenalidomide. Results: After 12 wk of lenalidomide treatment, erythroid responses were observed in all cases with an increase in hemoglobin levels of 2.7 ± 2.5 gdL (up to a mean 11.8 ± 1.9 gdL; P = 0.001). Before treatment, RPS14 expression levels were under-expressed in 15 patients with respect to normal controls. After 12 wk of lenalidomide treatment, all patients had an erythroid response. There was a significant increase in median RPS14 expression from baseline 0.01 (IQR 0.05-0.31) to 12 wk 204.71-fold (2.86-446.32; P <0.0001). Conclusions: These observations in the patient setting support the importance of RPS14 in the pathogenesis of MDS with del(5q).
AB - Background: Haploinsufficiency of the ribosomal protein S14 RPS14 gene, located in the common deleted region of chromosome 5q, is a potential causal factor of 5q- syndrome. Lenalidomide elicits high response rates and morphological improvements in myelodysplastic syndrome (MDS) patients with chromosome 5q deletion [del(5q)]. Methods: To further evaluate the role of RPS14, its transcription was tested in bone marrow cells from 17 patients with International Prognostic Scoring System defined Low- or Intermediate-1-risk MDS with del(5q) as a single or additional cytogenetic abnormality receiving treatment with lenalidomide. Results: After 12 wk of lenalidomide treatment, erythroid responses were observed in all cases with an increase in hemoglobin levels of 2.7 ± 2.5 gdL (up to a mean 11.8 ± 1.9 gdL; P = 0.001). Before treatment, RPS14 expression levels were under-expressed in 15 patients with respect to normal controls. After 12 wk of lenalidomide treatment, all patients had an erythroid response. There was a significant increase in median RPS14 expression from baseline 0.01 (IQR 0.05-0.31) to 12 wk 204.71-fold (2.86-446.32; P <0.0001). Conclusions: These observations in the patient setting support the importance of RPS14 in the pathogenesis of MDS with del(5q).
KW - del(5Q)
KW - lenalidomide
KW - myelodysplastic syndromes
KW - RPS14
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U2 - 10.1111/j.1600-0609.2010.01473.x
DO - 10.1111/j.1600-0609.2010.01473.x
M3 - Article
C2 - 20491881
AN - SCOPUS:77955812835
VL - 85
SP - 231
EP - 235
JO - European Journal of Haematology
JF - European Journal of Haematology
SN - 0902-4441
IS - 3
ER -