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Laboratory Indices in Patients with Positive and Borderline Flow-cytometry EMA-screening Test Results for Hereditary Spherocytosis

  • Author Footnotes
    ∗ Contributed equally
    David Azoulay
    Correspondence
    Corresponding author: David Azoulay, Ph.D. P.O.B 21 Nahariya, 22100 Israel, TEL: 972-4-910-7657, Fax: 972-4-910-7469
    Footnotes
    ∗ Contributed equally
    Affiliations
    Hematology Unit and Laboratories, Galilee Medical Center Nahariya

    Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel
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  • Author Footnotes
    ∗ Contributed equally
    Ilan Levov
    Footnotes
    ∗ Contributed equally
    Affiliations
    Hematology Unit and Laboratories, Galilee Medical Center Nahariya

    Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel
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  • Ety Shaoul
    Affiliations
    Hematology Unit and Laboratories, Galilee Medical Center Nahariya

    Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel
    Search for articles by this author
  • Amir Asher Kuperman
    Affiliations
    Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel

    Blood Coagulation Service and Pediatric Hematology Clinic, Galilee Medical Center, Nahariya, Israel
    Search for articles by this author
  • Author Footnotes
    ∗ Contributed equally
Published:January 13, 2022DOI:https://doi.org/10.1016/j.jpeds.2021.11.074

      Objective

      To evaluate laboratory Indices in patients with hereditary spherocytosis (HS), with positive and borderline flow-cytometry eosin-5-melamide (EMA)-bound red blood cells screening test.

      Study design

      We compared laboratory indices of 151 samples obtained from 139 different individual patients with negative, borderline, or positive EMA-test results. We also compared the clinical data of the patients in each EMA test results group.

      Results

      Borderline EMA-test results were obtained for 13 patients and were associated with more severe anemia, and lower reticulocyte count and reticulocyte production index, as compared with samples with positive EMA-test results. A receiving operator characteristic analysis identified MCHC < 32.5 g/dL as a cutoff, between positive/borderline and negative test results with 100% sensitivity. A higher prevalence of clinical markers typical of HS were found in patients with borderline or positive as compared with negative EMA test samples.

      Conclusions

      Based on laboratory data, borderline EMA-test results may be an indication of a more severe form of HS. Using MCHC as a cutoff may help predict and reduce negative EMA tests without compromising sensitivity. This finding needs to be further validated in other FC laboratories with a large EMA test sample pool.

      Keywords

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      References

        • King M.J.
        • Smythe J.S.
        • Mushens R.
        Eosin-5-maleimide binding to band 3 and Rh-related proteins forms the basis of a screening test for hereditary spherocytosis.
        British journal of haematology. 2004; 124: 106-113
        • Tse W.T.
        • Lux S.E.
        Red blood cell membrane disorders.
        British journal of haematology. 1999; 104: 2-13
        • Park S.H.
        • Park C.J.
        • Lee B.R.
        • Cho Y.U.
        • Jang S.
        • Kim N.
        • et al.
        Comparison study of the eosin-5'-maleimide binding test, flow cytometric osmotic fragility test, and cryohemolysis test in the diagnosis of hereditary spherocytosis.
        American journal of clinical pathology. 2014; 142: 474-484
        • Chari P.S.
        • Prasad S.
        Flow Cytometric Eosin-5'-Maleimide Test is a Sensitive Screen for Hereditary Spherocytosis.
        Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion. 2018; 34: 491-494
        • Girodon F.
        • Garcon L.
        • Bergoin E.
        • Largier M.
        • Delaunay J.
        • Feneant-Thibault M.
        • et al.
        Usefulness of the eosin-5'-maleimide cytometric method as a first-line screening test for the diagnosis of hereditary spherocytosis: comparison with ektacytometry and protein electrophoresis.
        British journal of haematology. 2008; 140: 468-470
        • Mackiewicz G.
        • Bailly F.
        • Favre B.
        • Guy J.
        • Maynadie M.
        • Girodon F.
        Flow cytometry test for hereditary spherocytosis.
        Haematologica. 2012; 97 (author reply e50-1): e47
        • Bianchi P.
        • Fermo E.
        • Vercellati C.
        • Marcello A.P.
        • Porretti L.
        • Cortelezzi A.
        • et al.
        Diagnostic power of laboratory tests for hereditary spherocytosis: a comparison study in 150 patients grouped according to molecular and clinical characteristics.
        Haematologica. 2012; 97: 516-523
        • Huisjes R.
        • Makhro A.
        • Llaudet-Planas E.
        • Hertz L.
        • Petkova-Kirova P.
        • Verhagen L.P.
        • et al.
        Density, heterogeneity and deformability of red cells as markers of clinical severity in hereditary spherocytosis.
        Haematologica. 2020; 105: 338-347
        • van Vuren A.
        • van der Zwaag B.
        • Huisjes R.
        • Lak N.
        • Bierings M.
        • Gerritsen E.
        • et al.
        The Complexity of Genotype-Phenotype Correlations in Hereditary Spherocytosis: A Cohort of 95 Patients: Genotype-Phenotype Correlation in Hereditary Spherocytosis.
        HemaSphere. 2019; 3: e276
        • King M.J.
        • Zanella A.
        Hereditary red cell membrane disorders and laboratory diagnostic testing.
        International journal of laboratory hematology. 2013; 35: 237-243
        • Liao L.
        • Xu Y.
        • Wei H.
        • Qiu Y.
        • Chen W.
        • Huang J.
        • et al.
        Blood cell parameters for screening and diagnosis of hereditary spherocytosis.
        Journal of clinical laboratory analysis. 2019; 33e22844