RESUMO
BACKGROUND: High risk myelodysplastic syndrome has various clinical courses and refractoriness to various therapies. It is important to analyze clinical characteristics and therapeutic responses in high risk myelodysplastic syndrome. METHODS: Sixty nine cases of primary high risk myelodysplastic syndrome at diagnosis were enrolled in this study at Kyungpook National University Hospital and Taegu Hyosung- Catholic University Hospital from January 1987 to June 1996. We have investigated the clinical characteristics and therapeutic outcomes after low dose cytarabine chemotherapy. RESULTS: 1) The median age of the patients was 48 years. Male to female ratio was 2.1:1. The each numbers of RAEB, CMML and RAEB-T patients were 38, 11 and 20, respectively. 2) The most common chief complaint was dyspnea on exertion. General weakness, fever and dizziness were also observed. The most common physical finding was pallor. 3) The peripheral blood findings showed anemia in 65 cases (94.2%), thrombocytopenia in 64 cases (92.8%), leukopenia in 32 cases (46.4%) and pancytopenia in 26 cases (37.7%). 4) Twenty two cases transformed to acute myelogenous leukemia during the follow-up periods. Chemotherapy was done in 18 cases among 22 cases of transformed acute myelogenous leukemia. Complete remission was achieved in 3 cases (16.7%), partial remission in 4 cases (22.2%) and no response in 11 cases (61.1%). 5) Forty seven cases were treated by low dose cytarabine chemotherapy. Complete response was achieved in 11 cases (23.4%), partial response in 13 cases (27.7%) and no response in 23 cases (48.9%). Median duration of complete response was 12 weeks. 6) We made score system, which based on Sanz score and Gattermann score, according to age, hemoglobin, platelet and bone marrow blast. Overall survival was higher in group A (score or = 6). Complete response of low dose cytarabine chemotherapy was higher in group A than group B but overall survival according to low dose cytarabine chemotherapy was not different in group A and group B. CONCLUSION: Low dose cytarabine chemotherapy was not effective in survival benefit. Score system according to prognostic factors was important to predict therapeutic response and prognosis. In the future, more intensive therapeutic plan and analysis of prognostic factors should be considered.