EMA结合试验检测的红细胞膜带3蛋白缺失程度与遗传性球形红细胞增多症临床表现型的关系

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.