Original Article

Leukemia (2008) 22, 544–550; doi:10.1038/sj.leu.2405076; published online 20 December 2007

Comparison of hypoplastic myelodysplastic syndrome (MDS) with normo-/hypercellular MDS by International Prognostic Scoring System, cytogenetic and genetic studies

T-C Huang1,2, B-S Ko1, J-L Tang1,2, C Hsu1, C-Y Chen1, W Tsay1, S-Y Huang1, M Yao1, Y-C Chen1, M-C Shen1, C-H Wang1 and H-F Tien1

1Division of Hemato-Oncology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan

Correspondence: Professor H-F Tien, Division of Hemato-Oncology, Department of Internal Medicine, National Taiwan University Hospital, no. 7, Chung-Shan South Road, Taipei 100, Taiwan, ROC. E-mail: hftien@ntu.edu.tw

2These authors contributed equally to this work.

Received 15 August 2007; Revised 23 October 2007; Accepted 19 November 2007; Published online 20 December 2007.

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Abstract

The differences in clinical features and prognosis between hypoplastic myelodysplastic syndrome (h-MDS) and normo-/hypercellular MDS (NH-MDS) remain unsettled. In this study, the characteristics of 37 h-MDS patients and 152 NH-MDS patients were compared. Peripheral-blood white blood cell counts and bone marrow blast percentage were lower in h-MDS patients than in NH-MDS patients (P=0.012 and 0.016, respectively). Refractory anemia (RA) was predominant (56.8%) in h-MDS, whereas RA with excess of blast (RAEB) was most common (44.7%) in NH-MDS. Chromosomal abnormalities -7/7q- occurred less frequently in h-MDS patients than in NH-MDS patients (0 vs 18.3%, P=0.022). There was no significant difference in the prevalence of mutations of RAS, AML1, JAK2, PTPN11, FLT3/ITD, and hypermethylation of SOCS1 and SHP1 between these two groups. International Prognostic Scoring System (IPSS) was ideal for predicting prognoses in h-MDS patients (P=0.002). In low- or intermediate-1 (Int-1)-risk MDS patients, h-MDS patients had a superior survival than NH-MDS patients (P=0.01). In conclusion, distinct from NH-MDS, h-MDS patients have different patterns of hemogram, distribution of French–American–British subtypes, cytogenetic changes and prognoses. IPSS is applicable in h-MDS as in NH-MDS. In patients with low- or Int-1-risk MDS, h-MDS patients have a better prognosis than NH-MDS patients.

Keywords:

MDS, cytogenetic, genetic, epigenetic, IPSS

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