Korean J Hematol 2004; 39(3):
Published online September 30, 2004
© The Korean Society of Hematology
만성골수증식질환의 임상양상
권정미, 이순남, 이경은, 윤수진, 남승현, 문영철, 성주명
이화여자대학교 의과대학 내과학교실
Clinical Features of Chronic Myeloproliferative Disease
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.
Keywords Chronic myeloproliferative disease, Philadelphia chromosome negative
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Korean J Hematol 2004; 39(3): 141-148
Published online September 30, 2004
Copyright © The Korean Society of Hematology.
만성골수증식질환의 임상양상
권정미, 이순남, 이경은, 윤수진, 남승현, 문영철, 성주명
이화여자대학교 의과대학 내과학교실
Clinical Features of Chronic Myeloproliferative Disease
Jung Mi Kwon, Soon Nam Lee, Kyoung Eun Lee, Su Jin Yoon, Seung Hyun Nam, Yeung, Chul Mun, Chu, Myong Seong
Department of Internal Medicine, Ewha Womans University College of Medicine, Seoul, Korea
Abstract
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.
Keywords: Chronic myeloproliferative disease, Philadelphia chromosome negative
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