遺傳性球型紅血球增多症(HS)是一種紅血球膜結構上的遺傳性異常,傳統上以紅血球脆性試驗進行篩檢,此項試驗非常的耗時、費力,且至少須取得9 ml的全血。EMA是一種螢光染劑,會與紅血球膜蛋白band 3上的Lys-430形成共價性鍵結;HS病患band 3蛋白數量會有下降的狀況,造成EMA螢光強度下降。為增進HS病患的篩檢能力,我們標準化流式細胞儀偵測EMA的步驟,透過ROC curve取得正常人與病患的cut-off值,並且分析HS病患分別在EMA試驗與脆性試驗的結果表現。總結來說,與脆性試驗相較,EMA試驗擁有較佳的靈敏度及專一性,操作簡易,數小時內便可得到結果。EMA試驗僅需少量的全血檢體,也適合在新生兒需要篩檢時使用。
Hereditary spherocytosis (HS) is a hereditary disorder of membrane structure on red blood cells. Traditional laboratory screening for HS is osmotic fragility test, which is labor-intensive and time-consuming requiring at least 9 ml of whole blood. EMA is a fluorescence dye, which binds covalently Lys-430 on the band 3 RBC membrane protein. The decrease of band 3 protein in HS patient results in the lower EMA fluorescence intensity. To improve the HS screening, we standardize the protocol of EMA flow cytometry. Set up a cut-off value through ROC curve to discriminate the normal individuals and HS patients. We also compare the performance between the EMA flow cytometry and OF test in a series of HS patients. Compared to the OF test, the EMA test has a better sensitivity and specificity, is easier to practice, and the results are available in hours. The EMA test needs small volume of whole blood, more suitable for neonatal screening.
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