中文摘要：

目的 探讨骨髓涂片有核红细胞糖原染色（PAS染色）在骨髓增生异常综合征（MDS）患者骨髓发育异常血细胞形态学判断、诊断、鉴别诊断中的意义.方法 回顾性分析406例MDS、67例巨幼细胞贫血（MegA）、76例缺铁性贫血（IDA）、207例非重型再生障碍性贫血（NSAA）、144例免疫性血小板减少性紫癜（ITP）、50例阵发性睡眠性血红蛋白尿症（PNH）、50例急性红白血病（AEL）患者的骨髓涂片有核红细胞PAS染色结果及MDS患者的其他相关实验室检查结果,并进行统计学分析.结果 MDS组有核红细胞PAS染色阳性检出率（53.0%）与NSAA组（14.5%）、ITP组（27.1%）、PNH组（16.0%）、AEL组（84.0%）比较差异均有统计学意义（P值均为0.000）,但与MegA组（46.3%）、IDA组（40.8%）比较差异无统计学意义（P值分别为0.310和0.052）.MDS组有核红细胞PAS染色阳性率（中位数,M=1%）及阳性积分（M＇=2）低于AEL组（M=8%,M＇=17）,高于NSAA组（M=0%,M＇=0）、ITP组（M=0%,M＇=0）、PNH组（M=0%,M＇=0）、MegA组（M=0%,M＇=0）、IDA组（M=0%,M＇=0）（P值均＜0.05）.有核红细胞PAS染色阳性率和阳性积分在除AEL外所有对照组中诊断MDS的最佳临界值（cut-off）分别为0.5%和0.5,其诊断敏感性60.8%,特异性74.4%.将MDS患者按有核红细胞PAS染色结果分为PAS阳性组和PAS阴性组,PAS阳性组较阴性组骨髓涂片红系比例（E）高,HGB水平低,平均红细胞体积（MCV）小,平均红细胞血红蛋白含量（MCH）、平均红细胞血红蛋白浓度（MCHC）低（P值均＜0.05）.PAS阳性组的骨髓巨核细胞总数、淋巴样小巨核细胞数及小巨核细胞占巨核细胞比例均高于PAS阴性组（P值均＜0.05）.外周血中性粒细胞碱性磷酸酶阳性指数（NALP）PAS阳性组低于PAS阴性组（P=0.000）.伴异常染色体核型的MDS患者有核红细胞PAS染色阳性检出率、阳性率、阳性积分均高于染色体核型正常MDS?

英文摘要：

Objective To investigate the implications of erythroblasts periodic acid-Schiff （PAS）stain for myelodysplastic syndromes （MDS） dyserythropoiesis, diagnosis and differential diagnosis. Methods PAS stain of bone marrow （BM） erythroblasts in 406 MDS pateints, 207 non-severe aplastic anemia （NSAA）, 144 immune thrombocytopenic purpura （ITP）, 67 megaloblastic anemia （MegA）, 76 iron deficiency anemia （IDA）, 50 paroxysmal nocturnal hemoglobinuria （PNH）, and 50 acute erythroid leukemia （AEL） as well as some related laboratory parameters in MDS patients were analyzed retrospectively. Results PAS-positive detection rate was significantly higher in MDS （53.0%） than in NSAA （14. 5%）, ITP （27.1%） and PNH （16.0%）, but was significantly lower in MDS than in AEL （84. 0%） （all P =0.000）. There was no significant difference in PAS-positive detection between MDS and MegA （46.3%）, or MDS and IDA （40.8 %） （P = 0.310, 0. 052, respectively）. Erythroblasts PAS-positive rate （Median, M =1%） and PAS-positive scores （M＇ =2） was significantly lower in MDS than in AEL （M =8%; M＇ = 17）,and significantly higher than in NSAA（M =0%; M＇ =0）, ITP（M =0%; M＇ =0）, PNH（M =0%; M＇ =0）, MegA （M = 0%; M＇ = 0）, and IDA （M = 0%; M＇ = 0） （all P 〈 0.05）. The cut-off value of PAS-positive rate and score for distinguishing MDS from the other groups except AEL were 0. 5% and 0. 5, with a sensitivity and specificity of 60.8% and 74.4%, respectively. For MDS patients, the percentage of BM erythroid cells was significantly higher in PAS-positive group than in PAS-negative group （P 〈 0.05）, and so were megakaryocyte count, lymphocyte-like micromegakaryocyte count and percentage of micromegakaryocyte （P =0.002, 0. 000, 0. 000, respectively）. HGB、MCV、MCH and MCHC were significantly lower in PASpositive group （all P 〈 0. 05）, and so was the neutrophil alkaling phosphatase （NALP） （P = 0.000）. PASpositive detection rate, p