282例原发性骨髓增生异常综合征诊断和分型的前瞻性临床研究

目的总结原发性骨髓增生异常综合征（MDS）诊断和分型的临床经验，提高诊断效率。方法采用前瞻性方法收集连续性样本，对上海市中美联合白血病协作组282例MDS患者进行综合诊断，同时采用FAB和WHO分型进行诊断。结果MDS中位发病年龄为56岁，50～59岁和70～79岁病例最多，分别占20％和25％。67％的患者外周血中出现幼稚粒细胞，48％出现幼稚红细胞。FAB—RA和WHO—RCMD患者比例较高，约占68％，高于欧美国家。染色体异常率为31．2％，低于欧美国家，但与日本相近。根据临床资料结合外周血和骨髓涂片细胞形态学检查诊断MDS的符合率为89％；将上述方法联合骨髓活检（平行试验）诊断MDS符合率可达94％；如将临床资料、细胞形态学、骨髓病理和细胞遗传学四者联合，其诊断符合率达97％。结论该组MDS患者在分型、发病年龄、全血细胞减少比例、染色体异常率等方面与日本报道相近，与欧美国家有不同。骨髓涂片细胞形态学、骨髓病理检查、细胞遗传学检查相结合可以极大提高MDS的诊断率。

Prospective clinical study of diagnosis and classification for 282 cases with primary myelodysplastic syndrome

Objective To summarize the diagnostie experience of pfimarty myelodysplastie syndromes (MDS)in order to improve tbe diagnostic level.Methods Using prospective cohort studv to collect consec- utive samples.Diagnoses of 282 MDS eases were defined aceording to FAB and WHO classification.Results The median age at MDS onset was 56.19.5% and 24.5% of eases occurred at age 50-59 and 70-79, respectively.Presence of immature granuloeytes and erythroblasts in peripheral blood (PB) were found in 67% and 48% of the MDS patients,respectively.The percentage (68%) of FAB-HA and WHO-RCMD pa- tients in China is relatively high compared to that in western eountries.The abnormality of chromosome (31.2%),was lower than that in the West,and similar to that in Japan.Eighty niue percent of the MDS pa- tients could be diagnosed on cell morphology in PB and bone marrow (BM) aspirate.Ninety four pereent of MDS could be diagnosed by combination of BM aspirate and core biopsy.Ninety seven percent of the patients could be recognized by combination of bone marrow aspirate,core biopsy and eytogenetics.Conclusions The subtypes and clinical features of Chinese MDS patients were somewhat different from the West,but simi- lar to that in Japan.Diagnostic efficiency can be extremely improved by combination of bone marrow aspirate, core biopsy and eytogenetics.