Comparison of hypoplastic myelodysplastic syndrome (MDS) with normo-/hypercellular MDS by International Prognostic Scoring System, cytogenetic and genetic studies.
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.
Huang
T-C
TC
Division of Hemato-Oncology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.
Ko
B-S
BS
Tang
J-L
JL
Hsu
C
C
Chen
C-Y
CY
Tsay
W
W
Huang
S-Y
SY
Yao
M
M
Chen
Y-C
YC
Shen
M-C
MC
Wang
C-H
CH
Tien
H-F
HF
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000753:Anemia, Refractory / Q000453:epidemiology; D000754:Anemia, Refractory, with Excess of Blasts / Q000453:epidemiology; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D019175:DNA Methylation; D004252:DNA Mutational Analysis; D006801:Humans; D006965:Hyperplasia; D007223:Infant; D007958:Leukocyte Count; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012720:Severity of Illness Index; D016019:Survival Analysis