Reliability of EMA Binding Test in the Diagnosis of Hereditary Spherocytosis in Italian Patients

Reliability of EMA Binding Test in the Diagnosis of Hereditary Spherocytosis in Italian Patients
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DOI:
10.1159/000322253
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发表时间:
2011-01-01
期刊:
影响因子:
2.4
通讯作者:
Rigano, Paolo
Rigano, Paolo
中科院分区:
医学4区
文献类型:
--
作者:
D'Alcamo, Elena;Agrigento, Veronica;Rigano, Paolo

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The eosin-5-maleimide (EMA) binding test has been described for the diagnosis of HS [9–14]. This method is based on the degree of fluorescence of red blood cells after incubation with EMA dye, which reacts covalently with Lys 430 on the first extracellular loop of band 3 protein. It also interacts with Rh protein, Rh glycoprotein and CD 47 [15].The EMA binding test has been shown to be an effective tool for diagnosis of HS in patients because it overcomes several difficulties concerning execution times, high costs of testing methods, amount of blood required, possibility of performing the EMA binding test several days after the blood sampling and the availability of methods only in specialized laboratories. This method only requires small quantities of blood (5 μ l of packed red cells) and, in contrast to other tests, is rapid and quite simple: the analysis can be performed on red blood cells stored at 4 C for several days. Our laboratory has found a high rate of false negative results obtained for mild HS when using the osmotic fragility, acid glycerol lysis test, and the Pink test. The aim of our study was to use the EMA binding test to establish a cut-off mean channel fluorescence (MCF) value for dis-