Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells
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 Asia...
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Online Access: | http://dx.doi.org/10.1155/2015/451861 |
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doaj-8119c0c3c73745418832034b7b3013852020-11-24T21:00:34ZengHindawi LimitedBioMed Research International2314-61332314-61412015-01-01201510.1155/2015/451861451861Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood CellsShin-ichiro Suemori0Hideho Wada1Hidekazu Nakanishi2Takayuki Tsujioka3Takashi Sugihara4Kaoru Tohyama5Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, JapanDepartment of Hematology, Kawasaki Medical School, Kurashiki, JapanDepartment of Hematology, Kawasaki Medical School, Kurashiki, JapanDepartment of Laboratory Medicine, Kawasaki Medical School, Kurashiki, JapanDepartment of Hematology, Kawasaki Medical School, Kurashiki, JapanDepartment of Laboratory Medicine, Kawasaki Medical School, Kurashiki, JapanFlow 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.http://dx.doi.org/10.1155/2015/451861 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Shin-ichiro Suemori Hideho Wada Hidekazu Nakanishi Takayuki Tsujioka Takashi Sugihara Kaoru Tohyama |
spellingShingle |
Shin-ichiro Suemori Hideho Wada Hidekazu Nakanishi Takayuki Tsujioka Takashi Sugihara Kaoru Tohyama Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells BioMed Research International |
author_facet |
Shin-ichiro Suemori Hideho Wada Hidekazu Nakanishi Takayuki Tsujioka Takashi Sugihara Kaoru Tohyama |
author_sort |
Shin-ichiro Suemori |
title |
Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells |
title_short |
Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells |
title_full |
Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells |
title_fullStr |
Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells |
title_full_unstemmed |
Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells |
title_sort |
analysis of hereditary elliptocytosis with decreased binding of eosin-5-maleimide to red blood cells |
publisher |
Hindawi Limited |
series |
BioMed Research International |
issn |
2314-6133 2314-6141 |
publishDate |
2015-01-01 |
description |
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. |
url |
http://dx.doi.org/10.1155/2015/451861 |
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