Korean J Hematol 1997; 32(3):

Published online September 30, 1997

© The Korean Society of Hematology

골수이형성 증후군의 예후 및 임상분석

김준환, 이경희, 현명수

연세대학교 의과대학 내과학교실

Evaluation of Clinical Characteristics and Prognostic Factors in the Patients of Myelodysplastic Syndrome

Jun Hwan Kim, Kyung Hee Lee, Myung Soo Hyun

Department of Internal Medicine, Youngnam University, College of Medicine, Taegu, Korea

Abstract

Background: Myelodysplastic syndrome(MDS) is a disorder of the hematopoietic pluripotent stem cell. The treatment modality and its prognosis were based on the FAB classification. But, because there is a variation of the clinical course, we evaluated the clinical characteristics and prognostic factors of MDS with score system.
Method : We analyzed age, FAB classification, symptoms, peripheral blood and bone marrow findings of MDS patients for evaluation of clinical course and prognosis.
Results:
1) Age & Sex : Male to female ratio was 19:11, and age distribution was mainly over 5th decade(77%).
2) Subtype : The most common subtype was RAEB(43%), followed by RA(23%),RAEBT(17%), CMML(10%), and RARS(6.7%).
3) Initial symptom : The most common symptom was exertional dyspnea, followed by general weakness, headache, dizziness, and febrile sensation.
4) Peripheral blood Anemia(< 10g/dL) was noted in 90%, leukopenia(<2.5×10 9/L) in 36.6%, and thrombocytopenia(<100×10 9/L) in 73.3%.
5) Bone marrow finding : Hypocellularity was noted in 23%, normocellularity in 13%, hypercellularity in 63%. Bone marrow fibrosis was noted in 1 case of CMML.
6) Duration of mean follow-up period was 12.3 months and mean survival rate was 17.4 months .
7) The score of Sanz and Gattermann in the patient with RAEB and RAEBT were significantly higher than those of the patients with RA and RARS. Transformation to AML was seen in 5 cases with group C of Gattermann score.
8) With anabolic steroid therapy in 10 cases of RA & RARS, 2 cases were clinically improved, and with low dose Ara-C therapy in 5 cases, one case in RARS and another in RAEB were completely remixed but the latter convered to AML after 16 months.
Conclusion: The patients with RA and RARS have longer survival rate than the patients with other types of MDS. The score system of Bournemouth, Sanz and Gattermann were useful in evaluating the prognosis of MDS, being Gattermann score more valuable than those of Bournmouth and Sanz to predict the prognosis of MDS.

Keywords Myelodysplastic syndrome; Score system; Prognostic factor;

Article

Korean J Hematol 1997; 32(3): 398-408

Published online September 30, 1997

Copyright © The Korean Society of Hematology.

골수이형성 증후군의 예후 및 임상분석

김준환, 이경희, 현명수

연세대학교 의과대학 내과학교실

Evaluation of Clinical Characteristics and Prognostic Factors in the Patients of Myelodysplastic Syndrome

Jun Hwan Kim, Kyung Hee Lee, Myung Soo Hyun

Department of Internal Medicine, Youngnam University, College of Medicine, Taegu, Korea

Abstract

Background: Myelodysplastic syndrome(MDS) is a disorder of the hematopoietic pluripotent stem cell. The treatment modality and its prognosis were based on the FAB classification. But, because there is a variation of the clinical course, we evaluated the clinical characteristics and prognostic factors of MDS with score system.
Method : We analyzed age, FAB classification, symptoms, peripheral blood and bone marrow findings of MDS patients for evaluation of clinical course and prognosis.
Results:
1) Age & Sex : Male to female ratio was 19:11, and age distribution was mainly over 5th decade(77%).
2) Subtype : The most common subtype was RAEB(43%), followed by RA(23%),RAEBT(17%), CMML(10%), and RARS(6.7%).
3) Initial symptom : The most common symptom was exertional dyspnea, followed by general weakness, headache, dizziness, and febrile sensation.
4) Peripheral blood Anemia(< 10g/dL) was noted in 90%, leukopenia(<2.5×10 9/L) in 36.6%, and thrombocytopenia(<100×10 9/L) in 73.3%.
5) Bone marrow finding : Hypocellularity was noted in 23%, normocellularity in 13%, hypercellularity in 63%. Bone marrow fibrosis was noted in 1 case of CMML.
6) Duration of mean follow-up period was 12.3 months and mean survival rate was 17.4 months .
7) The score of Sanz and Gattermann in the patient with RAEB and RAEBT were significantly higher than those of the patients with RA and RARS. Transformation to AML was seen in 5 cases with group C of Gattermann score.
8) With anabolic steroid therapy in 10 cases of RA & RARS, 2 cases were clinically improved, and with low dose Ara-C therapy in 5 cases, one case in RARS and another in RAEB were completely remixed but the latter convered to AML after 16 months.
Conclusion: The patients with RA and RARS have longer survival rate than the patients with other types of MDS. The score system of Bournemouth, Sanz and Gattermann were useful in evaluating the prognosis of MDS, being Gattermann score more valuable than those of Bournmouth and Sanz to predict the prognosis of MDS.

Keywords: Myelodysplastic syndrome, Score system, Prognostic factor,

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