![]() The OGE curves of all the hemoglobinopathies are shown in the FIGURE Significantly decreased (p<0.01) parameters have been found for EImax in HbC, HbSS and BT, Omin in HbC, HbS, HbSS, HbSC and BT, Ohyper in all hemoglobinopathies and AUC in HbC, HbSS and BT. Results: Exception made of HbE, in all the structural hemoglobinopathies and thalassemia the tail of the osmoscan curve shifts to the left suggesting the existence of a decreased osmotic fragility and dehydration. The area under the curve (AUC) or the distance between the starting point in the hypo-osmolar region (Omin) and an ending point in the hyper-osmolar region (Ohyper) that partially depends of red cell deformability The RBC hydration status (Ohyper) that depends of the intracellular viscosity or hemoglobin concentration (MCHC) and 4. The osmotic fragility (Omin) that depends on the surface-to-volume ratio (S/V), 3. The elongation index (EImax), that depends mostly of the cytoskeleton mechanics 2. OGE provided by the osmoscan module of the Laser Optical Rotational Red Cell Analyser (LoRRca) MaxSis provides four main parameters1. Methods: The patients included in this study have been studied via a stepwise process including RBC morphology, high performance liquid chromatography (HPLC) complemented by Hb electrophoresis, and the measurement of common RBC enzyme activities.When necessary, the genetic diagnosis of hemoglobinopathies was performed by t-NGS. In addition this study aims to improve our knowledge of the contribution of red cell deformability, osmotic fragility and intracellular viscosity to the pathophysiology of hemolysis especially when these disorders are a cause of rare anemia Classical examples of this situation are HS, unstable hemoglobinopathies, sickle cell disease and homozygous b-thalassemia (BT) but up to now, the OGE experience with structural hemoglobinopathies and thalassemia is scarceĪims: We have evaluated the OGE profiles in 70 patients with different hemoglobinopathies, both structural and thalassemia, with the aim to analyse their usefulness for the early diagnosis of these disorders either individual or in coinheritance with other hereditary RBC defects. Impairment of red cell deformability leading to the decrease of red cell survival rate, is the common trait of hereditary haemolytic anemias and some hemoglobinopathies In general this is the consequence of the abnormal cell shape, increased rigidity or dehydration. ![]() Background: Next Generation Ektacytometry is up to now one of the best complementary diagnostic tool for hereditary rare anemias due to red cell defects. ![]()
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