필라델피아 음성 만성골수증식성질환의 임상고찰

Abstract

Background : Philadephia chronosome negative chronic myeloproliferative disorde(CMPD) is a clonal disorder which includes polycythemia vera, essential thrombocythemia and idiopathic myelofibrosis. CMPD has chronic course and different clinical features. Especially CMPD accompany vascular complications. We evaluated the clinical features, complications, treatment and effect of treatment of CMPD Methods : We retrospectively reviewed the medical records of patients with CMPD between December 2002 and December 2006. Result : Eighteen patients were identified. The mean age of 18 CMPD patients was 64 years(range, 40-83) and male to female ratio was 1:2. Clinical manifestations were abdominal discomfort(22%), aquagenic pruritis(22%), skin phletora(22%), bleeding(16%), thrombosis(11%) and dizziness(11%). Treatment with hydrea was most frequent during clinical course of CMPD. Complication of disease itself and treatment was not frequent except bleeding and thrombotic event. Conversion to acute leukemia was none. Conclusions : CMPD is a chronic disease and desirable effect result from DNA biosynthesis inhibitor and cyclic nucleotide phosphodiesterase inhibitor.