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        골수이형성증후군의 임상혈액학적 고찰

        손상균 ( Son Sang Gyun ),정병천 ( Jeong Byeong Cheon ),김광원 ( Kim Gwang Won ),이재태 ( Lee Jae Tae ),이규보 ( Lee Gyu Bo ),황기석 ( Hwang Gi Seog ) 대한내과학회 1990 대한내과학회지 Vol.38 No.6

        N/A Myelodysplastic syndrome is a primary hematologic disease characterized by ineffective hematopoiesis with peripheral blood cytopenias. The authors conducted a clinicohematologic observation of 55 cases of MDS from July 1983 to June 1988. The result are as follows. The peak age incidence was in the fifth decade, and the male-to-female ratio was nearly 1:1. RA was observed most frequently. Thereafter RAEB-T, RAEB, RARS and CMML were observed, respectively. The chief complaints of the patients were exertional dyspnea and fatigability by anemia. Physical findings showed pallor (85.5%), purpura (40.0%), retinal hemorrhage (38.2%), hepatomegaly (18.2%), lymphadenopathy (14.5%), icteric sclera (12.7%) and splenomegaly (10.9%). The common infection sites were oral (38.0%), systemic (sepsis: 20:0%) and lung (14.5%). The main hematologic changes were pancytopenia (56.4%) and then anemia and thro.mbocytopenia, anemia and leukopenia, anemia and thrombocytopenia and leukocytosis, anemia only was present in 20.0%, 10.9%, 7. 5% and 5.4%, respectively. The laboratory findings showed increased serum iron (48.1%), decreased serum ferritin (43.1%), normal LAP scores (89.1%), prolonged plasm iron disappearance time (48.3%), shortened PID (6.9%), increased LDH (56.3%), increased vitamin B12 (29.5%) and decreased folic acid (4.5%). The bone marrow finding showed nearly hypercellular (38.2%) or normocellular (45.5%), along with variable morphological abnormalities, such as dyserythropoiesis, dysgranulopoiesis and dysmegakaryopoiesis. Cytogenetic studies showed abnormal chromosomal findings in five out of 13 cases. With combination therapy using androgen, pyridoxine and corticosteroid in 29 cases of RA and RARS, 31% of patients were clinically improved. And with low dose Ara-C therapy in 15 cases of RAEB, CMML and RAEBT, 26.6% of cases were improved. In the cases of RAEB and RAEB-T, nine cases of RAEB and RAEB-T converted to an other subtype of MDS or leukemia within one to six months. Nine cases expired (sepsis in five cases and CVA in four cases) during the observation period.

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