New-Generation Ektacytometry Study of Red Blood Cells in Different Hemoglobinopathies and Thalassemia
نویسندگان
چکیده
Next-generation ektacytometry provided by the osmoscan module of Laser Optical Rotational Red Cell Analyser (LoRRca) MaxSis is, so far, one best complementary diagnostic tools for congenital rare anaemias due to red blood cell defects. Osmotic gradient (OGE) is currently considered gold standard diagnosis membrane disorders, especially hereditary spherocytosis (HS). Impairment deformability, leading a decrease in survival rate, common trait haemolytic anaemias; general, it consequence an abnormal shape, increased rigidity or dehydration. Up now, next-generation has been mainly used differential membranopathies, but experience with structural hemoglobinopathies and thalassemia still scarce. However, recently, many new forms therapy are being developed treatment hemoglobinopathies, particularly sickle-cell disease ?-thalassemia; clinical interest increasing should be further explored. Here, we have evaluated OGE profiles LoRRca ektacytometer 96 patients different both thalassemia, aim analysing their usefulness early these disorders either individually co-inheritance other RBC In addition, this study aims improve our knowledge contribution osmotic fragility intracellular viscosity physiopathology haemolysis, when cause anaemia. From study, conclude that profile provides information on deformability hydration homeostasis may contribute better understanding decreased hemolysis which present some patients.
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ژورنال
عنوان ژورنال: Thalassemia Reports
سال: 2023
ISSN: ['2039-4357', '2039-4365']
DOI: https://doi.org/10.3390/thalassrep13010007