伴环状铁粒幼细胞增多的骨髓增生异常综合征17例实验室特点分析

Analysis of laboratory characteristics of myelodysplastic syndrome with ringed sideroblasts in 17 cases

目的分析伴环状铁粒幼细胞增多的骨髓增生异常综合征（MDS）的实验室特点。方法对17例伴有环状铁粒幼细胞增多的MDS患者的实验室资料进行回顾性分析。结果17例患者中男12例（70．6％），女5例（29．4％）；中位年龄65岁。外周血3系减少8例，MCV（101．6±12．2）n，其中≥100．0fl13例。骨髓增生明显活跃10例，粒系发育异常细胞比例0．01—0．26，红系发育异常细胞比例0．10～0．33，巨核系发育异常细胞比例0-v0．27。环状铁粒幼细胞16％～40％（23．6±8．0）％。10例有染色体核型分析结果的患者中异常7例（70．0％）。生存时间1～84个月，中位时间17个月。结论伴有环状铁粒幼细胞增多的MDS多发于老年男性，起病多表现为贫血所致的乏力，骨髓增生程度好，病态造血程度轻，环状铁粒幼细胞比例增高但以〈30％者为多，染色体畸变发生率高，以大细胞性贫血多见，生存期较长，病死率低。

Objective To study the laboratory characteristics of myelodysplastic syndrome （MDS） with ringed sidero- blasts. Methods The laboratory data of 17 MDS patients with ringed sideroblasts were reviewed and analyzed. Results In 17 patients, there were 12 male cases （70.6%）, 5 female cases （29.4%）, the median age was 65 years and ≥60 years old in 10 cases （58.8%）. The 8 cases shows pancytopenia and the MCV was （101.6 ± 12.2） fl,which was more than 100.0 fl in 13 cases. The ratio of dysgranulomonopoiesis ranged from 0.01 - 0.26, dyserythropoiesis ranged from 0.10 - 0.33, anddysmegakaryocytopoiesis ranged from 0 - 0.27. The ringed sideroblasts was 16% - 40% （23.6 ± 8.0） %. There were 7 ca- ses have abnormal karyotypes in 10 cases with chromosome karyotype results, accounted for 70.0% ,the survival time was 1 -84 months the median time was 17 months. Conclusion Myelodysplastic syndrome with ringed sideroblasts multiple in the elderly male people and the onset of disease was manifested as anemia caused by fatigue. The bone marrow hyperplasia was well developed, and dysplasia in lower degree. Ring sideroblasts percentage increased but 〈 30% were for many, the high in- cidence of chromosomal aberrations in large cell anemia common, longer survival, the fatality rate is low.