Toward the harmonization of result presentation for the eosin-5′-maleimide binding test in the diagnosis of hereditary spherocytosis

Percentage of hyperdense cells: Automated parameter to hereditary spherocytosis screening

“…However, we found a raised cut-off point when compared to other studies [11,12]. Probably, the limited number of patients with HS, and the fact of their clinical condition be classified between moderate to severe [4] may explain the results found. Accordingly, the cut-off point of 6.4% might not be used in the trait or mild HS, making it necessary for more studies to include HS individuals with all disease conditions (both homozygous and heterozygous).…”

“…Currently, it is not indicated due to its low sensitivity, leading to false negative results with the presence of other causes of anemia [2]. The eosin-5-maleimide (EMA) by binding test is performed by flow cytometry (FC) and has a sensitivity of 99%, but the reactants used are unstable and costly [4][5][6]. Flowcytometric osmotic fragility test (FCM OF) is another method by FC, which evaluates the resistance of RBC to lysis after being exposed to deionized water.…”

Disease-modifying influences of coexistent G6PD-deficiency, Gilbert syndrome and deletional alpha thalassemia in hereditary spherocytosis: A report of three cases

“…Visual evaluation of the tracing may reveal tailing as in our Case 3, representing a subset of highly fragile red cells that are conditioned by the spleen [10]. The EMA-dye binding test has rapidly established itself as a very useful adjunct, especially in such diagnostically ambiguous situations [11].…”

CD44 as a Potential Screening Marker for Preliminary Differentiation Between Congenital Dyserythropoietic Anemia Type II and Hereditary Spherocytosis

“…This option (involving an invasive sampling procedure) is only considered when other types of anemia with similar presentation and phenotype have been excluded in a diagnostic work-up. Furthermore, some CDAII can give positive test results in the Eosin-5 0 -maleimide (EMA) binding test (a screening test for HS) (19,20). Some CDAII patients are misdiagnosed to have hereditary spherocytosis (HS) due to occasional spherocytes on the blood smear (17).…”