title: Analysis of Hereditary Elliptocytosis with Decreased Binding of Eosin-5-maleimide to Red Blood Cells.
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.
authors.affiliation: Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Suemori
authors.affiliation: Department of Hematology, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Hideho
authors.initials: H
authors.lastname: Wada
authors.affiliation: Department of Hematology, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Nakanishi
authors.affiliation: Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Tsujioka
authors.affiliation: Department of Hematology, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Sugihara
authors.affiliation: Department of Laboratory Medicine, Kawasaki Medical School, Kurashiki, Japan.
authors.forename: Kaoru
authors.initials: K
authors.lastname: Tohyama
mesh_terms: D016022:Case-Control Studies; D004612:Elliptocytosis, Hereditary / Q000378:metabolism*; D004801:Eosine Yellowish-(YS) / Q000031:analogs & derivatives* / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism*; D005434:Flow Cytometry / Q000379:methods; D006801:Humans; D012680:Sensitivity and Specificity
chemical_list: C033531:eosin maleimide; D004801:Eosine Yellowish-(YS)