Comparison of hypoplastic myelodysplastic syndrome (MDS) with normo-/hypercellular MDS by International Prognostic Scoring System, cytogenetic and genetic studies
Journal
Leukemia
Journal Volume
22
Journal Issue
3
Pages
544-550
Date Issued
2008
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.
SDGs
Other Subjects
anthracycline derivative; antineoplastic agent; CD135 antigen; cytarabine; Janus kinase 2; protein tyrosine phosphatase; protein tyrosine phosphatase SHP 1; Ras protein; suppressor of cytokine signaling 1; transcription factor RUNX1; acute granulocytic leukemia; adolescent; adult; aged; allogeneic hematopoietic stem cell transplantation; article; child; chromosome 7; chromosome 7q; chromosome aberration; clinical feature; controlled study; diagnostic value; differential diagnosis; disease classification; female; gene mutation; high risk patient; human; human tissue; infant; International Prognostic Scoring System; leukocyte count; low drug dose; major clinical study; male; myelodysplastic syndrome; prediction; priority journal; prognosis; refractory anemia; refractory anemia with excess blasts; statistical significance; survival rate
Publisher
Nature Publishing Group
Type
journal article