Abstract
Flow cytometric test for analyzing the eosin-5-maleimide (EMA) binding to red blood cells has been believed to be a specific method for diagnosing hereditary spherocytosis (HS). However, it has been reported that diseases other than HS, such as hereditary pyropoikilocytosis (HPP) and Southeast Asian ovalocytosis (SAO), which are forms in the category of hereditary elliptocytosis (HE), show decreased EMA binding to red blood cells. We analyzed EMA binding to red blood cells in 101 healthy control subjects and 42 HS patients and obtained a mean channel fluorescence (MCF) cut-off value of 36.4 (sensitivity 0.97, specificity 0.95). Using this method, we also analyzed 12 HE patients. Among them, four HE patients showed the MCF at or below the cut-off value. It indicates that some HE patients have decreased EMA binding to red blood cells. Two of these four HE patients were classified as common HE, and two were spherocytic HE with reduced spectrin. This study demonstrates that, in addition to patients with HPP or SAO, some HE patients have decreased EMA binding to red blood cells.
Highlights
Eosin-5-maleimide (EMA) is a fluorochrome that primarily binds to band 3 of red blood cell membrane proteins
Among patients with red blood cell membrane abnormalities other than hereditary spherocytosis (HS), some patients with hereditary pyropoikilocytosis (HPP) and Southeast Asian ovalocytosis (SAO), which are forms of hereditary elliptocytosis (HE), show decreased EMA binding to red blood cells [7, 8]
Using receiver operating characteristic (ROC) curves of mean channel fluorescence (MCF) values for EMA binding to red blood cells, the cutoff value in HS patients was 36.4
Summary
Eosin-5-maleimide (EMA) is a fluorochrome that primarily binds to band 3 of red blood cell membrane proteins. Among patients with red blood cell membrane abnormalities other than HS, some patients with hereditary pyropoikilocytosis (HPP) and Southeast Asian ovalocytosis (SAO), which are forms of hereditary elliptocytosis (HE), show decreased EMA binding to red blood cells [7, 8]. There have been few studies examining EMA binding to red blood cells in the HE patients, other than HPP and SAO, and the results are variable [1, 3,4,5]. We analyzed EMA binding to red blood cells in 12 HE patients observed in our department and examined the relationship between the types of HE and EMA binding to red blood cells
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
