Laboratory Indices in Patients with Positive and Borderline Flow Cytometry Eosin-5-Maleimide-Screening Test Results for Hereditary Spherocytosis.

Autor: Azoulay D; Hematology Unit and Laboratories, Galilee Medical Center, Nahariya, Israel; Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. Electronic address: davidA@GMC.gov.il., Levov I; Hematology Unit and Laboratories, Galilee Medical Center, Nahariya, Israel; Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel., Shaoul E; Hematology Unit and Laboratories, Galilee Medical Center, Nahariya, Israel; Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel., Kuperman AA; Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel; Blood Coagulation Service and Pediatric Hematology Clinic, Galilee Medical Center, Nahariya, Israel.
Jazyk: angličtina
Zdroj: The Journal of pediatrics [J Pediatr] 2022 Apr; Vol. 243, pp. 142-145. Date of Electronic Publication: 2022 Jan 14.
DOI: 10.1016/j.jpeds.2021.11.074
Abstrakt: Objective: To evaluate laboratory indices in patients with hereditary spherocytosis, 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 compared with samples with positive EMA-test results. A receiving operator characteristic analysis identified mean corpuscular hemoglobin concentration of <32.5 g/dL as a cut-off, between positive/borderline and negative test results with 100% sensitivity. A higher prevalence of clinical markers typical of hereditary spherocytosis was found in patients with borderline or positive 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 hereditary spherocytosis. Using mean corpuscular hemoglobin concentration as a cut-off may help predict and reduce negative EMA tests without compromising sensitivity. This finding needs to be further validated in other flow cytometry laboratories with a large EMA test sample pool.
(Copyright © 2022 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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