骨髓增生异常综合征75例临床分析

目的　进一步认识骨髓增生异常综合征 (MDS)的临床、血液学和骨髓病态造血的特点。方法　对 1995年 1月～ 2 0 0 2年 1月确诊的 75例MDS患者的一般情况、临床和实验室检查特点进行总结分析。结果　 75例MDSFAB分型难治性贫血 (RA) 46例 ,难治性贫血伴环状铁粒幼细胞增多 (RAS) 4例 ;按WHO 1997新诊断标准真正RA(包括RAS 2例 )仅 8例 ,有 3 9例属于“难治性血细胞减少伴多系增生异常 (RCMD)”范畴。 72例 ( 96%)和 3 9例 ( 5 2 %)患者分别有贫血和全血细胞减少 ,2 2 .6%患者在外周血片可见到有核红细胞 ,16%见到幼稚粒细胞 ,2 .6%见到小巨核细胞 ;T细胞亚群显示明显异常 ,骨髓中红系祖细胞体外培养集落明显减或缺如。 2 5例患者染色体检查 12例检出异常核型 ,以5q 和t( 8;2 1)平衡易位较常见 ;复杂核型多见于MDS RAEB或RAEB t患者。骨髓中红、粒巨幼样变分别占 2 7.3 %和 2 4%,小巨核细胞检出率占 3 0 .7%,骨髓活检示幼红细胞岛和未成熟前体粒细胞位置异常 (ALIP)现象分别占 3 3 .3 %和 5 2 .8%。RA和RAS采用雄激素为主的治疗 ,62 %( 3 1/5 0 )血红蛋白有所提高 ,RAEB和RAEB t大多转化急性白血病。结论　应加强对WHO分型新标准的认识 ,骨髓病态造血特别是ALIP和小巨核的检出对诊断具有重要价值并可能反映

Clinical analysis of 75 cases of myelodysplastic syndrome

Objective To investigate and analysis clinically 75 cases of myelodysplastic syndrome (MDS), including the characters of hematology and dysplasia in bone marrow.Methods The generalcondition, clinical and laboratory features, andtherapy of these patients from 1995.1-2002.1 were studied. Results Acording to FAB subtypes, 46 cases were diagnosed refractoy anemia (RA), 4 cases. RA with ringed sideroblastics (RAS) By WHO new criterias in MDS, the real RA only was 8 cases(including RAS 2 cases), 39 cases, which belong to RA by FAB criterias, were diagnosed refractory cytopenia of multilineage dysplasia (RCMD). 72cases (96%) and 39 cases (52%) developed anemia and pancytopenia respectively atdiagnosis. 22.6%,16% and 2.6% cases were found erythroblasts, promyelocytes andmicromegakaryocytes in blood smears. Subtypes of T lymphocytes showed highly abnormalities, the quantity of BFU E's culture in vitro were decreased. Twelve out of 25 patients had karyotypic abnormalities, 5q and t(8;21) translocation were often seen. Complicated karyotypic abnormalities always associated with MDS RAEB or RAEB t. Bone marrow aspirate smears showed megaloblastoid erthropoiesis and granulocytopoiesis in 27.3% and 24% patients, micromegakaryocytes were 30.7%. Bone marrow biopsy specimens showed the islands of erythroblasts and abnormal localization of immature precursors(ALIP) in 33.3% and 52.8% patients. The level of hemoglobin in 62%(31/50) of patients with RA and RAS were increased during androgen therapy,the majority of RAEB and RAEB t patients were thansformatedto acute leukemia.Conclusion A new insight should be madeto recognize WHO new criterias of MDS, bone marrow dysplasia especially ALIP and micromegakaryocytes are important for diagnosis of MDS, and may be reflectingprognosis.