https://scholars.lib.ntu.edu.tw/handle/123456789/589661
Title: | Distinct mutation profile and prognostic relevance in patients with hypoplastic myelodysplastic syndromes (h-MDS) | Authors: | CHI-YUAN YAO HSIN-AN HOU Lin, Tzung-Yi CHIEN-CHIN LIN WEN-CHIEN CHOU Tseng, Mei-Hsuan Chiang, Ying-Chieh Liu, Ming-Chih Liu, Chia-Wen Kuo, Yuan-Yeh SHANG-JU WU Liao, Xiu-Wen Lin, Chien-Ting BOR-SHENG KO Chen, Chien-Yuan SZU-CHUN HSU Li, Chi-Cheng SHANG-YI HUANG MING YAO JIH-LUH TANG WOEI TSAY Liu, Chieh-Yu HWEI-FANG TIEN |
Keywords: | bone marrow hypocellularity; gene mutation; myelodysplastic syndromes; prognosis; revised international prognostic scoring system;Bone marrow hypocellularity; Gene mutation; Myelodysplastic syndromes; Prognosis; Revised international prognostic scoring system | Issue Date: | 27-Sep-2016 | Journal Volume: | 7 | Journal Issue: | 39 | Source: | Oncotarget | Abstract: | 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. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/589661 | ISSN: | 1949-2553 | DOI: | 10.18632/oncotarget.11050 | SDG/Keyword: | adolescent; adult; aged; Article; ASXL1 gene; cancer incidence; cancer prognosis; cancer survival; controlled study; DNMT3A gene; EZH2 gene; female; gene expression profiling; gene mutation; human; hypoplastic myelodysplastic syndrome; International Prognostic Scoring System; major clinical study; male; malignant transformation; mutational analysis; myelodysplastic syndrome; oncogene; overall survival; progression free survival; RUNX1 gene; thrombocyte count; TP53 gene; blood; cohort analysis; disease free survival; dna mutational analysis; genetics; incidence; leukocyte; metabolism; middle aged; mutation; myelodysplastic syndrome; prognosis; Taiwan; very elderly; young adult; Adolescent; Adult; Aged; Aged, 80 and over; Cohort Studies; Disease-Free Survival; DNA Mutational Analysis; Female; Humans; Incidence; Leukocytes; Male; Middle Aged; Mutation; Myelodysplastic Syndromes; Prognosis; Taiwan; Young Adult |
Appears in Collections: | 醫學院附設醫院 (臺大醫院) |
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