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Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)

Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic malignancies. Although most MDS patients have normal or increased BM cellularity (NH-MDS), some have hypocellular BM (h-MDS). The reports concerning the differences in genetic alterations between h-MDS and NH-MDS patients are l...

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Autores principales: Yao, Chi-Yuan, Hou, Hsin-An, Lin, Tzung-Yi, Lin, Chien-Chin, Chou, Wen-Chien, Tseng, Mei-Hsuan, Chiang, Ying-Chieh, Liu, Ming-Chih, Liu, Chia-Wen, Kuo, Yuan-Yeh, Wu, Shang-Ju, Liao, Xiu-Wen, Lin, Chien-Ting, Ko, Bor-Shen, Chen, Chien-Yuan, Hsu, Szu-Chun, Li, Chi-Cheng, Huang, Shang-Yi, Yao, Ming, Tang, Jih-Luh, Tsay, Woei, Liu, Chieh-Yu, Tien, Hwei-Fang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Impact Journals LLC 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5325355/
https://www.ncbi.nlm.nih.gov/pubmed/27527853
http://dx.doi.org/10.18632/oncotarget.11050
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author Yao, Chi-Yuan
Hou, Hsin-An
Lin, Tzung-Yi
Lin, Chien-Chin
Chou, Wen-Chien
Tseng, Mei-Hsuan
Chiang, Ying-Chieh
Liu, Ming-Chih
Liu, Chia-Wen
Kuo, Yuan-Yeh
Wu, Shang-Ju
Liao, Xiu-Wen
Lin, Chien-Ting
Ko, Bor-Shen
Chen, Chien-Yuan
Hsu, Szu-Chun
Li, Chi-Cheng
Huang, Shang-Yi
Yao, Ming
Tang, Jih-Luh
Tsay, Woei
Liu, Chieh-Yu
Tien, Hwei-Fang
author_facet Yao, Chi-Yuan
Hou, Hsin-An
Lin, Tzung-Yi
Lin, Chien-Chin
Chou, Wen-Chien
Tseng, Mei-Hsuan
Chiang, Ying-Chieh
Liu, Ming-Chih
Liu, Chia-Wen
Kuo, Yuan-Yeh
Wu, Shang-Ju
Liao, Xiu-Wen
Lin, Chien-Ting
Ko, Bor-Shen
Chen, Chien-Yuan
Hsu, Szu-Chun
Li, Chi-Cheng
Huang, Shang-Yi
Yao, Ming
Tang, Jih-Luh
Tsay, Woei
Liu, Chieh-Yu
Tien, Hwei-Fang
author_sort Yao, Chi-Yuan
collection PubMed
description Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic malignancies. Although most MDS patients have normal or increased BM cellularity (NH-MDS), some have hypocellular BM (h-MDS). The reports concerning the differences in genetic alterations between h-MDS and NH-MDS patients are limited. In this study, 369 MDS patients diagnosed according to the WHO 2008 criteria were recruited. h-MDS patients had lower PB white blood cell and blast counts, and lower BM blast percentages, than those with NH-MDS. h-MDS was closely associated with lower-risk MDS, defined by the International Prognostic Scoring System (IPSS) and revised IPSS (IPSS-R). IPSS-R could properly predict the prognosis in h-MDS (P<0.001) as in NH-MDS patients. The h-MDS patients had lower incidences of RUNX1, ASXL1, DNMT3A, EZH2 and TP53 mutations than NH-MDS patients. The cumulated incidence of acute leukemic transformation at 5 years was 19.3% for h-MDS and 40.4% for NH-MDS patients (P= 0.001). Further, the patients with h-MDS had longer overall survival (OS) than those with NH-MDS (P= 0.001), and BM hypocellularity remains an independent favorable prognostic factor for OS irrespective of age, IPSS-R, and gene mutations. Our findings provide evidence that h-MDS indeed represent a distinct clinico-biological subgroup of MDS and can predict better leukemia-free survival and OS.
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spelling pubmed-53253552017-03-23 Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS) Yao, Chi-Yuan Hou, Hsin-An Lin, Tzung-Yi Lin, Chien-Chin Chou, Wen-Chien Tseng, Mei-Hsuan Chiang, Ying-Chieh Liu, Ming-Chih Liu, Chia-Wen Kuo, Yuan-Yeh Wu, Shang-Ju Liao, Xiu-Wen Lin, Chien-Ting Ko, Bor-Shen Chen, Chien-Yuan Hsu, Szu-Chun Li, Chi-Cheng Huang, Shang-Yi Yao, Ming Tang, Jih-Luh Tsay, Woei Liu, Chieh-Yu Tien, Hwei-Fang Oncotarget Research Paper Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic malignancies. Although most MDS patients have normal or increased BM cellularity (NH-MDS), some have hypocellular BM (h-MDS). The reports concerning the differences in genetic alterations between h-MDS and NH-MDS patients are limited. In this study, 369 MDS patients diagnosed according to the WHO 2008 criteria were recruited. h-MDS patients had lower PB white blood cell and blast counts, and lower BM blast percentages, than those with NH-MDS. h-MDS was closely associated with lower-risk MDS, defined by the International Prognostic Scoring System (IPSS) and revised IPSS (IPSS-R). IPSS-R could properly predict the prognosis in h-MDS (P<0.001) as in NH-MDS patients. The h-MDS patients had lower incidences of RUNX1, ASXL1, DNMT3A, EZH2 and TP53 mutations than NH-MDS patients. The cumulated incidence of acute leukemic transformation at 5 years was 19.3% for h-MDS and 40.4% for NH-MDS patients (P= 0.001). Further, the patients with h-MDS had longer overall survival (OS) than those with NH-MDS (P= 0.001), and BM hypocellularity remains an independent favorable prognostic factor for OS irrespective of age, IPSS-R, and gene mutations. Our findings provide evidence that h-MDS indeed represent a distinct clinico-biological subgroup of MDS and can predict better leukemia-free survival and OS. Impact Journals LLC 2016-08-04 /pmc/articles/PMC5325355/ /pubmed/27527853 http://dx.doi.org/10.18632/oncotarget.11050 Text en Copyright: © 2016 Yao et al. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Paper
Yao, Chi-Yuan
Hou, Hsin-An
Lin, Tzung-Yi
Lin, Chien-Chin
Chou, Wen-Chien
Tseng, Mei-Hsuan
Chiang, Ying-Chieh
Liu, Ming-Chih
Liu, Chia-Wen
Kuo, Yuan-Yeh
Wu, Shang-Ju
Liao, Xiu-Wen
Lin, Chien-Ting
Ko, Bor-Shen
Chen, Chien-Yuan
Hsu, Szu-Chun
Li, Chi-Cheng
Huang, Shang-Yi
Yao, Ming
Tang, Jih-Luh
Tsay, Woei
Liu, Chieh-Yu
Tien, Hwei-Fang
Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title_full Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title_fullStr Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title_full_unstemmed Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title_short Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS)
title_sort distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-mds)
topic Research Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5325355/
https://www.ncbi.nlm.nih.gov/pubmed/27527853
http://dx.doi.org/10.18632/oncotarget.11050
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