65例骨髓增生异常综合征的临床及实验室分析

本研究探讨骨髓增生异常综合征（MDS）的临床及实验室检查特点。对65例MDS临床资料进行回顾性总结。65例MDS患者的FAB分型为：RA27例，RAS1例，RAEB33例，RAEB—T3例，CMML1例；中位年龄66岁（19—89岁），65例MDS中继发性MDS6例。在研究中对患者进行了骨髓细胞学检查，骨髓活检和染色体检查。结果表明，经骨髓细胞学检查为病态造血者64例（98．5％），其中3系病态造血者21例（32．3％），2系病态造血33例（50．8％），1系病态造血10例（单纯红系8例占12．3％，单纯粒系2例占3．1％）。骨髓活检38例，其中6例有幼稚前体细胞异常定位（ALIP）。染色体检查49例患者，其中29例检测出有异常核型（59．2％），且以数量异常为主，高危型MDS（RAEB或RAEB—T）的核型异常发生率高于低危型MDS（RA，RAS），继发性MDS高于原发性MDS；在这49例患者中15例转为急性白血病（30．61％），20例染色体正常组中3例（15％）转化为AML，而染色体异常组29例中有12例（41．4％）转化为AML。随访中位时间为35个月（2个月-106个月），染色体核型正常组中位生存期为26．8个月，异常组中位生存期为8个月。结论：我国MDS患者发病年龄较国外略年轻；高危MDS患者染色体畀常发生率高于低危MDS，染色体改变与MDS的白血病转化密切相关，核型异常组的白血病转化率较核型正常组高，生存时间短。

Analysis on Laboratory and Clinical Characteristics in 65 Cases of Myelodysplastic Syndrome

The aim of this study was to gain more insight into the understanding of myelodysplastic syndrome in the clinical and laboratory features. The clinical data of 65 patients with MDS were reviewed and analysed. According to FAB criteria, 65 patients were classified as follows： 27 patients with RA, 1 pateint with RAS ,33 patients with RAEB ,3 patients with RAEB-T, and 1 patient with CMML. The median age of them was 66 years old （range 19 -89 years）, and 6 patients had a history of toxic exposure （ secondary MDS ）. The bone marrow smears, bone marrow biopsy and cytogenetic examinations were performed in this study. The results showed that dysplasia was found in 64 patients examined with bone marrow smears （98.5%）, among them trilineage dysplasia in 21 patients （32.3%）, bilineage dysplasia in 33 patients （50.8%）, only erythroid dysplasia in 8 cases （12.3%） and 2 patients （3. 1% ）only with myeloid dysplasia. The bone marrow biopsy was performed in 38 patients, abnormal localization of immature precursor （ALIP） occured in 6 cases. 29 patients had abrormal karytypes, accounting for 59.2% of the 49 patients subjected cytogenetic examination. The abnormal chromosome was the major cytogenetic abnormality, which ocurred more often in secondary MDS and the patients with RAEB or RAEB-T. Among the 49 patients who had received cytogenetic examination, 15 patients transformed into AML with the incidence of 30.61%, but only 3 out of 20 patients in the group of normal chromosome transformed into AML （ 15% ）, while 12 out of 29 patients in the group of abnormal karyotypes transformed into AML （41.4%）. The median time of following up was 35 months （range 2 - 106 months）. The median survival time was 26.8 months and 8 months in the patients with normal karyotype and chromosome aberrations respectively. In conclusion, the incidence of MDS in our country is younger than that in Western countries, the rate of abrormal chromosome in high risk MDS is higher than that in low risk MDS. Meanwhile, those who have the change of chromosome are related to the transformation of MDS into AML and have shorter survival time than those MDS patients with normal karyotypes.