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目的建立多指标综合诊断骨髓增生异常综合征(MDS)诊断标准。方法以前瞻性方法验证从回顾性研究中得出的综合诊断MDS的8项指标。观察中国医学科学院血液病医院2000年1月至2004年6月住院MDS患者,其中多指标诊断组38例,同期对照组79例,既往对照组123例,对比分析3组患者转化为白血病的情况。结果随访期间多指标诊断组18例转化为白血病,转白率为47.37%,中位转白时间6(1～33)个月。同期对照组18例转化为白血病,转白率为22.78%(与多指标诊断组相比,P<0.01),中位转白时间6(1～33)个月。既往对照组16例转化为白血病,转白率为13.01%(与多指标诊断组相比P<0.01),中位转白时间5(1～23)个月。多指标转白的相对危险度:骨髓原始粒细胞和单核细胞≥0.020的相对危险度(RR)为9.11,髓系细胞分化指数≥1.8的RR为6.50,有淋巴样微巨核RR=4.55,外周血中出现幼稚粒、单核细胞RR=4.40,骨髓有核红细胞糖原染色阳性RR=4.26,染色体核型异常RR=2.87,骨髓细胞体外培养粒、单核细胞系集簇与集落比值≥4.0的RR为2.14。结论多指标综合诊断较全面反映MDS恶性造血克隆的生物学本质,能更准确地诊断MDS患者。
Objective To establish a multi-index diagnosis of myelodysplastic syndrome (MDS) diagnostic criteria. Methods To prospectively validate eight indicators of MDS diagnosed by retrospective study. Observe the MDS patients from January 2000 to June 2004 in the Hematology Hospital of Chinese Academy of Medical Sciences. Among them, 38 patients in the multi-index diagnosis group, 79 in the control group in the same period and 123 in the previous control group were compared to analyze the leukemia in 3 groups . Results During the follow-up period, 18 patients with multi-index diagnosis turned into leukemia, the conversion rate was 47.37%, and the median transit time was 6 (1 ~ 33) months. In the same period, 18 cases were transformed into leukemia in the control group, the rate of white conversion was 22.78% (P <0.01 compared with the multi-index diagnosis group), and the median transit time was 6 (1 to 33) months. In the control group, 16 cases were converted to leukemia, the conversion rate was 13.01% (P <0.01 compared with the multi-index diagnosis group), and the median transit time was 5 (1-23) months. The relative risk of multi-indicator translocation: the relative risk (RR) of bone marrow myeloblasts and monocytes≥0.020 was 9.11, the RR of myeloid differentiation≥1.8 was 6.50, the lymphoid micro-megakaryocyte RR was 4.55, In the peripheral blood, there were immature granule, monocyte RR = 4.40, bone marrow erythrocyte glycogen staining positive RR = 4.26, chromosome karyotype RR = 2.87, bone marrow cell culture in vitro, monocyte cell cluster and colony ratio ≥ The RR for 4.0 is 2.14. Conclusion The multi-index comprehensive diagnosis comprehensively reflects the biological nature of MDS malignant hematopoietic clonality and can diagnose MDS patients more accurately.