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Hyun Woo Lee, Hyeoung Il Kim, Jae Myoung Choi, Seok Yun Kang, Jun Ho Jang, Joon Seong Park, Jin Hyuk Choi, Ho Yeong Lim, Hugh Chul Kim |
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부산대학교 의과대학 내과학교실 |
| 원저 : 골수이형성증후군의 분류에 따른 임상상 및 예후 (Original Articles : Clinical characteristics and prognosis according to the classification of myelodysplastic syndrome) |
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이현우, 김형일, 최재명, 강석윤, 장준호, 박준성, 최진혁, 임호영, 김효철 |
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| Abstract |
Background : Myelodysplastic syndromes (MDS) are clonal hematologic stem cell disorders
characterized clinically and morphologically by ineffective hematopoiesis. A consensus-defined
French-American-British (FAB) classification and International Prognostic Scoring System (IPSS)
for predicting outcome and planning therapy in MDS has been developed, but its prognostic value in
a large and independent series remains unproven. So we investigate clinical characteristics and
prognosis of MDS, according to French-American-British (FAB) classification and International
Prognostic Scoring System (IPSS).
Methods
: A retrospective analysis of 50 patients who were diagnosed as myelodysplastic
syndrome at Ajou University Hospital was performed from November, 1994 to April, 2003. The
patients with secondary MDS were excluded. All patients were classified according to the FAB
classification and calculated prognostic scores for IPSS. Patients were evaluated for clinical features
and for blood and bone marrow findings at the time of diagnosis, and were followed up for survival
and leukemic progression. Survival curves were based on the Kaplan-Meier method. All reported p
values less than or equal to 0.05 were regarded as stastistically significant.
Results
: The peak age was in the fifth decade and the male to female ratio was 1.5:1. RA (36%)
was observed most frequently. Thereafter, RAEB-t (26%), RAEB (24%), RARS (12%) and CMML
(2%) were observed, respectively. The initial symptoms on admission were fever (24%), dizziness
and headache (16%), general weakness (16%), hemorrhage (14%), dyspnea (12%), abdominal pain
(4%) and vomiting (4%). Cytogenetic studies were performed in 34 patients with MDS. They were
classified as good, intermediate, poor group by chromosome score of IPSS. The median survival was
16.4 months for the good group, 15 months for the intermediate, 10.3 months for the poor. The
median survival according to FAB classified groups were RA (33.8 mo), RARS (12.5 mo), RAEB
(16.4 mo), RAEB-t (6.7 mo) and CMML (1.3 mo). Survival according to IPSS scoring system were 67.2 months for low, 27.1 months for intermediate-1, 10.3 months for intermediate-2 and 6.0 months
for high groups. These data were statistically significant (p<0.05).
Conclusions
: In our experiencies, FAB and IPSS classification would be good predictors in
clinical outcomes. But, because of the heterogeneity of MDS, large multicenter studies will be
needed to define the issue of a new classification for these disorders.(Korean J Med 70:253-260,
2006)
Key Words : Myelodysplastic syndrome, Classification |
| Key Words:
Myelodysplastic syndrome, Classification |
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