标准型α-珠蛋白生成障碍性贫血患儿红细胞参数及网织红细胞的分群分析

A cluster analysis of erythrocyte index and reticulocyte in children with a-thalassemia trait

目的探讨红细胞参数及网织红细胞三分群参数在标准型α-珠蛋白生成障碍性贫血(α-地贫)患儿中的变化及意义。方法将经基因确诊的56例6～13岁标准型α-地贫患儿设为观察组,80例同一年龄阶段的健康儿童设为对照组,观察分析两组参数的变化。红细胞参数包括红细胞计数(RBC)、血红蛋白测定(Hb)、红细胞平均体积(MCV)、红细胞平均血红蛋白含量(MCH)、红细胞平均血红蛋白浓度(MCHC)、红细胞体积分布宽度(RDW-CV)和Mentzer指数(MCV/RBC);网织红细胞参数包括网织红细胞百分率(RET%)及三分群参数即低荧光强度网织红细胞百分率(LFR%)、中荧光强度网织红细胞百分率(MFR%)和高荧光强度网织红细胞百分率(HFR%),采用统计学方法比较两组间参数的差异。结果标准型α-地贫患儿红细胞参数的主要特征表现为小红细胞增多,Hb减低,MCV、MCH、MCHC均下降,RDW-CV轻度升高,Mentzer指数小于13。网织红细胞三分群表现为LFR%降低,MFR%和HFR%相应升高。除RET%外,观察组的其他各参数值与对照组比较差异均有统计学意义(P<0.01)。结论红细胞参数及网织红细胞三分群参数对地贫患儿的筛查及其鉴别诊断具有一定的临床应用价值,为进一步进行基因诊断提供了重要的参考依据。

Objective To investigate the change and significance of the erythrocyte index and three cluster re- ticulocyte parameters in children with thalassemia. Methods 56 children aged 6 13 with thalassemia who received the genetic diagnosis were chosen as a survey group, 80 healthy children with the same age were randomly chosen as a control group,and the change of the indexes for the two groups were observed and analyzed. The erythrocyte index included RBC, Hb, MCV, MCH, MCHC, RDW-CV, and MCV/RBC. The reticulocyte index included RET~ and three cluster parameters （LFR%,MFR~, HFR~）,and the index difference between the two groups was compared by the statistical method. Results The main character of the erythrocyte index in children with thalassemia was that the microcytosis increased and H b reduced, MCV, MVH and MCHC all declined, RDW-CV ascended slightly, MCV/ RBC was below 13, three cluster reticulocyte parameters represented that LFR% decreased, and both MFR~ and HFR~ ascended accordingly. And except RET~, there was significant difference about comparing other indexes of the survey group and the control one （P（0.01）. Conclusion The erythrocyte index and three cluster reticulocyte parameters had the clinical practical value for the screening,identification and diagnosis of children with thalassemia, and it provided the important reference proof for the further molecular diagnosis.