European journal of haematology

Low RPS14 expression in MDS without 5q - aberration confers higher apoptosis rate of nucleated erythrocytes and predicts prolonged survival and possible response to lenalidomide in lower risk non-5q- patients.

PMID 23506134


Haploinsufficiency of the ribosomal protein S14 (RPS14) has been identified as a causal factor in myelodysplastic syndrome (MDS) with isolated del (5q). This study was carried out to investigate RPS14 expression in MDS without 5q deletion and the role of lower expressed RPS14 plays in pathogenesis and the prognosis and lenalidomide-response predicting of non-5q-MDS. The expression level of RPS14 transcription was detected in 156 MDS patients without 5q-. The apoptosis of bone marrow (BM) nucleated erythrocytes was also analyzed. Furthermore, patient prognosis with lower or normal RPS14 expression was analyzed, and the role of RPS14 expression in lenalidomide-response prediction was evaluated. The reduced RPS14 expression occurred in 83 of 156 (53.2%) non-5q-patients. Patients with RPS14 lower expression presented higher platelet counts in the peripheral blood compared with RPS14 normal patients (P = 0.012). The lower RPS14 expression status was inversely correlated with increased apoptosis ratio in nucleated erythrocytes from BM (r = -0.54, P = 0.013). Patients with lower RPS14 expression have a higher 2-yr survival probability than normal RPS14 cases in the international prognosis scoring system (IPSS) lower risk group (90.8% vs. 71.7%; P = 0.018). A multivariate analysis showed RPS14 expression status was an independent predictor for survival in lower risk MDS patients without 5q deletion. Twelve patients were treated with lenalidomide. Five of seven patients achieved an erythroid response in the lower RPS14 expression group (5/7, 71.4%), compared with zero responses in the five normal RPS14 patients (P = 0.018). Lower RPS14 expression in MDS patients without 5q deletion is associated with increased apoptosis of nucleated erythrocytes in lower risk MDS. Additionally, lower RPS14 predicts prolonged survival and possible response to lenalidomide in lower risk MDS patients.