Clinical characteristics and prognosis according to the classification of myelodysplastic syndrome / 대한내과학회지

ABSTRACT

BACKGROUND: Myelodysplastic syndromes (MDS) are clonal hematologic stem cell disorders characterized clinically and morphologically by ineffective hematopoiesis. A consensus-defined French-American-British (FAB) classification and International Prognostic Scoring System (IPSS) for predicting outcome and planning therapy in MDS has been developed, but its prognostic value in a large and independent series remains unproven. So we investigate clinical characteristics and prognosis of MDS, according to French-American-British (FAB) classification and International Prognostic Scoring System (IPSS). METHODS: A retrospective analysis of 50 patients who were diagnosed as myelodysplastic syndrome at Ajou University Hospital was performed from November, 1994 to April, 2003. The patients with secondary MDS were excluded. All patients were classified according to the FAB classification and calculated prognostic scores for IPSS. Patients were evaluated for clinical features and for blood and bone marrow findings at the time of diagnosis, and were followed up for survival and leukemic progression. Survival curves were based on the Kaplan-Meier method. All reported p values less than or equal to 0.05 were regarded as stastistically significant. RESULTS: The peak age was in the fifth decade and the male to female ratio was 1.5:1. RA (36%) was observed most frequently. Thereafter, RAEB-t (26%), RAEB (24%), RARS (12%) and CMML (2%) were observed, respectively. The initial symptoms on admission were fever (24%), dizziness and headache (16%), general weakness (16%), hemorrhage (14%), dyspnea (12%), abdominal pain (4%) and vomiting (4%). Cytogenetic studies were performed in 34 patients with MDS. They were classified as good, intermediate, poor group by chromosome score of IPSS. The median survival was 16.4 months for the good group, 15 months for the intermediate, 10.3 months for the poor. The median survival according to FAB classified groups were RA (33.8 mo), RARS (12.5 mo), RAEB (16.4 mo), RAEB-t (6.7 mo) and CMML (1.3 mo). Survival according to IPSS scoring system were 67.2 months for low, 27.1 months for intermediate-1, 10.3 months for intermediate-2 and 6.0 months for high groups. These data were statistically significant (p<0.05). CONCLUSIONS: In our experiencies, FAB and IPSS classification would be good predictors in clinical outcomes. But, because of the heterogeneity of MDS, large multicenter studies will be needed to define the issue of a new classification for these disorders.