E Llaudet-Planas1,2, J L Vives-Corrons1,2, V Rizzuto2, P Gómez-Ramírez1, J Sevilla Navarro3, M T Coll Sibina4, M García-Bernal5, A Ruiz Llobet6, I Badell7, P Velasco-Puyó8, J L Dapena8, M M Mañú-Pereira1,2. 1. Red Cell Pathology Unit, Hospital Clínic de Barcelona, [Institut d'Investigacions Biomèdiques August Pi I Sunyer] IDIBAPS, [Universitat de Barcelona] UB, Barcelona, Spain. 2. Red Cell Pathology Unit, [Josep Carreras Leukemia Research Institute] IJC, [Universitat Autònoma de Barcelona] UAB, Badalona, Spain. 3. Servicio Hematología Pediátrica, [Hospital Infantil Universitario Niño Jesús] HIUNJ, [Fundación por la Investigación Biomédica] FIB, CIBERER, Madrid, Spain. 4. Pediatric Department, Hospital General de Granollers, Granollers, Spain. 5. Pediatric Hematology and Oncology Department, Hospital Universitari Mútua de Terrassa, Terrassa, Spain. 6. Pediatric Hematology and Oncology Department, Hospital Sant Joan de Déu de Barcelona, [University of Barcelona] UB, Barcelona, Spain. 7. Unidad de Hematología, Oncología y Trasplante Hematopoyético, Hospital Universitari de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona [UAB], Barcelona, Spain. 8. Pediatric Oncology and Hematology Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona [UAB], Barcelona, Spain.
Abstract
INTRODUCTION: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. METHODS: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques. The most robust osmoscan parameters for hereditary spherocytosis diagnosis were determined using receiver operating characteristic curve analysis. RESULTS: The best diagnostic criteria for hereditary spherocytosis were the combination of decreased minimal elongation index up to 3% and increased minimal osmolality point up to 5.2% when compared to the mean of controls. Using this established criterion, osmotic gradient ektacytometry reported a sensitivity of 93.85% and a specificity of 98.38% for the diagnosis of hereditary spherocytosis. CONCLUSION: Osmotic gradient ektacytometry is an effective diagnostic test for hereditary spherocytosis and enables its differential diagnosis with other red blood cell membrane diseases based on specific pathology profiles.
INTRODUCTION: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. METHODS: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques. The most robust osmoscan parameters for hereditary spherocytosis diagnosis were determined using receiver operating characteristic curve analysis. RESULTS: The best diagnostic criteria for hereditary spherocytosis were the combination of decreased minimal elongation index up to 3% and increased minimal osmolality point up to 5.2% when compared to the mean of controls. Using this established criterion, osmotic gradient ektacytometry reported a sensitivity of 93.85% and a specificity of 98.38% for the diagnosis of hereditary spherocytosis. CONCLUSION: Osmotic gradient ektacytometry is an effective diagnostic test for hereditary spherocytosis and enables its differential diagnosis with other red blood cell membrane diseases based on specific pathology profiles.
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