RESUMO
BACKGROUND: Philadephia chromosome negative chronic myeloproliferative disease (CMPD) is a clonal disorder which includes polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). CMPD has chronic course and different clinical features with low rate of conversion to leukemia. We evaluated the clinical features of CMPD. METHODS: Since 1990, 57 cases of CMPD (18 PV, 35 ET and 4 IMF) were analysed and their clinical characteristics, survival and manner of evolution were evaluated retrospectively. RESULTS: Median age of 57 CMPD patients was 61 (range, 14~90) years and male to female ratio was 1:0.8. Most common clinical manifestations were dizziness/weakness (38.6%), headache (21.2%), cardiovascular events (19.3%) and other symptoms. Treatment with hydroxyurea was most frequent during clinical course of CMPD. Anagrelide was introduced in 12 patients recently. Complication of disease itself and treatment was not frequent except bleeding (3 cases) and thrombotic event (10 cases). Conversion to acute lekemia was none. Ten year overall survival was 83.3% in PV, 60.1% in ET and 4 cases of IMF were all alive at the 6 year follow up. CONCLUSION: CMPD is a chronic disease and long term control is much improved but definitive treatment without complication should be further investigated.