Objective: To investigate the relationship between the eosin-5'-maleimide (EMA) binding test and the clinical severity of hereditary spherocytosis (HS). Methods: A total of 258 un-splenectomize HS patients were consecutively enrolled. Correlation of hemoglobin concentration, hemolytic parameters, compensating erythropoiesis and the EMA binding test were evaluated. Results: 258 (128 male and 130 female) patients were included in this study, including 91 compensatory hemolysis patients, 53 patients with mild anemia, 78 patients with moderate anemia and 36 patients with severe anemia. The median age at diagnosis was 23 (2-70) years. The median decreased fluorescence intensity of EMA binding test was 29.97% (16.09%-47.34%) and the average intensity was (29.70±6.28) % of 258 HS patients. The decreased EMA binding fluorescence intensity correlated with MCV (r=-0.343, P<0.001) and MCHC (r=0.223, P<0.001). There was no relationship between EMA fluorescence intensity and absolute reticulocyte count (r=0.080, P=0.198) , reticulocyte percentile (r=-0.015, P=0.813) , IBIL levels (r=-0.009, P=0.902) , HGB levels (r=-0.067, P=0.280). Evaluated as a quartile variable, EMA fluorescence intensity was not correlated with anemia severity (C=0.150, P=0.746). Conclusion: EMA binding test does not related to anemia levels and has no major clinical implications for disease severity in HS.
目的： 探讨伊红-5′-马来酰亚胺标记的流式细胞术（EMA结合试验）检测红细胞膜骨架带3蛋白缺失程度与遗传性球形红细胞增多症（hereditary spherocytosis，HS）临床表现型的关系。 方法： 分析258例未行脾切除术治疗的HS患者临床和实验室特征，评估EMA结合试验结果与贫血程度、溶血和造血代偿参数的关系。 结果： 258例HS患者中，男128例，女130例，中位年龄23（2~70）岁。代偿性溶血91例、轻度贫血53例、中度贫血78例、重度贫血36例。EMA结合试验荧光强度减低中位数为29.97%（16.09%~47.34%），平均数为（29.70±6.28）%。荧光强度减低程度与红细胞平均体积呈负相关（r=-0.343，P<0.001），与红细胞平均血红蛋白浓度呈正相关（r=0.223，P<0.001），与网织红细胞比例（r=-0.015，P=0.813）和绝对值（r=0.080，P=0.198）均无明显相关性，与血清间接胆红素水平无明显相关（r=-0.009，P=0.902），与HGB水平无明显相关性（r=-0.067，P=0.280）。按EMA标记缺失程度四分位区间分组，不同EMA标记缺失组与HS贫血严重程度分组亦无明显相关性（C=0.150，P=0.746）。 结论： EMA结合试验结果与HS贫血程度无关。.
Keywords: Anemia; Eosin-5′-maleimide; Spherocytosis, hereditary.