MDS-RA/RARS/RCMD患者外周血循环CD34^+细胞计数的临床价值

Peripheral blood circulating CD34^+ cell count in patients with MDS-RA/RARS/RCMD

目的探讨骨髓增生异常综合征(MDS)-难治性贫血(RA)/难治性贫血伴环形铁粒幼细胞增多(RARS)/难治性贫血伴多系异常(RCMD)患者外周血循环CD34^+细胞计数的临床价值。方法采用流式细胞仪检测19名造血干细胞供者(正常对照者)、52例MDS-RA/RARS/RCMD患者外周血循环CD34^+细胞百分比和计数。根据国际预后积分系统(IPSS)及世界卫生组织(WHO)分型预后积分系统(WPSS),将MDSRA/RARS/RCMD患者分为低危、中危Ⅰ、中危Ⅱ、高危及极低危、低危、中危、高危、极高危。评价外周血循环CD34^+细胞计数与MDS-RA/RARS/RCMD患者临床特点的相关性。结果 MDS-RA/RARS/RCMD患者外周血循环CD34^+细胞百分比和计数均高于正常对照者(P<0.01),外周血循环CD34^+细胞计数与患者性别、年龄、外周血血细胞减少程度、白细胞计数下降、幼稚前体细胞异常定位(ALIP)现象均无相关性(r<0.625,P>0.05),而与染色体核型异常、IPSS预后积分、WPSS预后积分、骨髓纤维化相关(r>0.995,P<0.01)。外周血循环CD34^+细胞计数>10.00×10~6/L的16例MDS-RA/RARS/RCMD患者中,有12例伴有复杂染色体核型异常,其中10例伴有7号染色体核型异常;有12例IPSS预后积分为中危Ⅱ;有13例WPSS预后积分为高危;9例伴有骨髓纤维化。36例外周血循环CD34^+细胞计数<10.00×10~6/L的患者均不伴有复杂染色体核型异常及7号染色体核型异常;所有患者IPSS预后积分均为低危或中危Ⅰ;除1例患者WPSS预后积分为高危外,其余患者的WPSS预后积分均为极低危、低危或中危;除1例患者伴有骨髓纤维化外,其余患者均不伴有骨髓纤维化。结论MDS-RA/RARS/RCMD患者外周血循环CD34^+细胞计数可出现异常增加,外周血循环CD34^+细胞计数检测有助于对MDS患者疾病危险度的进一步分层。

Objective To evaluate peripheral blood circulating CD34＋ cell count in patients with myelodysplasticsyndrome（MDS）-refractory anemia（RA）/refractory anemia with ring siderblasts（RARS）/refractorycytopenia with multilineage dysplasia（RCMD）. Methods A total of 52 MDS-RA/RARS/RCMD patients and 19hematopoietic stem cell donors （healthy controls） were enrolled,and their peripheral blood circulating CD34＋cell percentages and counts were determined by flow cytometry. They were classified into low risk, intermediate-Ⅰrisk,intermediate-Ⅱ risk,high risk subgroups and very low risk,low risk, intermediate risk,high risk, veryhigh risk subgroups according to the International Prognostic Scoring System （IPSS） and World Health Organization（WHO）-Based Prognostic Scoring System （WPSS）,respectively. The correlation of peripheral blood circulatingCD34＋ cell count with the clinical characteristics of MDS-RA/RARS/RCMD patients was analyzed. Results MDSRA/RARS/RCMD patients had higher percentage and count of peripheral blood circulating CD34＋ cells comparedwith healthy controls（P〈0.01）. There was no correlation between peripheral blood circulating CD34＋ cell count in MDS-RA/RARS/RCMD patients and patients＇ sex,age,cytopenia degree,white blood cell count decreasing andabnormal localization of immature precursor （ALIP）（r〈0.625,P〉0.05）,and there was correlation betweenperipheral blood circulating CD34＋ cell count and abnormal karyotype,IPSS score, WPSS score and myelofibrosis（r〉0.995,P〈0.01）. There were 16 MDS-RA/RARS/RCMD patients with peripheral blood circulating CD34＋ cellcount 〉10.00×106/L. Among them, there were 12 patients with complex karyotype abnormalities （10 patients withchromosome 7 abnormalities）. A total of 12 and 13 patients belonged to IPSS intermediate-Ⅱ risk and WPSS highrisk subgroups,respectively. There were 9 patients with myelofibrosis. Among 36 MDS-RA/RARS/RCMD patientswith peripheral blood circulating CD34＋ cell count 〈10.00×106/L,no patient had complex karyotype abnormalitiesor chromosome 7 abnormalities. Except for 1 patient in WPSS high risk subgroup,other patients were in IPSSlow,intermediate-Ⅰ risk subgroups and WPSS very low,low and intermediate risk subgroups. Only 1 patient hadmyelofibrosis. Conclusions The count of peripheral blood circulating CD34＋ cells increases in MDS-RA/RARS/RCMD patients,which can be further used for the classification of MDS risk.