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: Shin-ichiro Suemori, Hideho Wada, Hidekazu Nakanishi, Takayuki Tsujioka, Takashi Sugihara, Kaoru Tohyama
• Journal: BioMed research international (Biomed Res Int) Vol. 2015 Pg. 451861 ( 2015) ISSN: 2314-6141 [Electronic] United States
• PMID: 26557672 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• eosin maleimide
• Eosine Yellowish-(YS)

Topics

• Case-Control Studies
• Elliptocytosis, Hereditary (metabolism)
• Eosine Yellowish-(YS) (analogs & derivatives, metabolism)
• Erythrocytes (metabolism)
• Flow Cytometry (methods)
• Humans
• Sensitivity and Specificity