Assessment of eritrocyte mechanics in patients with sickle cell anemia without transfusion, hemoglobin H, hemoglobin D and thalassemia intermedia

Hemoglobinopathies corrupt the function of the erythrocytes along with different psychopathological mechanisms. Thalassemia intermedia, sickle cell anemia, alpha thalassemia and hemoglobin D are different hemoglobinopathies. The main purpose of this study is to assess mechanical features of erythrocytes in hemoglobin H, thalassemi intermedia, sickle cell anemia and hemoglobin D cases. In our study, we included 2 hemoglobin H patients, 4 thalassemi intermedia patients, 3 sickle cell anemia patients, 5 hemoglobin D carriers, 1 hemoglobin D patient and a control group of 17 healthy people. Since the numbers of hemoglobin H, thalassemia intermedia ve Hb S patients were inadequate in terms of statistical assessment, these were gathered under a group named HIS. Cases with hemoglobin D and hemoglobin D carriers were gathered under hemoglobin D. Hemogram parameters of each case were assessed. With the aim of verifying hemoglobin H diagnosis in hemoglobin H cases, hemoglobin H coloring was made and erythrocytes were examined under microscope. LORCA ektacytometer was used in the assessment of erythrocyte mechanics. In order to study on erythrocytes of patient groups, a special attention was given to those patients who were not subjected to transfusion, were taken into account. The blood samples, after being suspended with the help of PVPs with 100 cp viscosity, were embedded between two glass cylinders in ektacytometer. Shear stress at different type and periods were applied into erythrocytes over three stages. At the first stage, 9 shear stress between 0,3 to 50 Pa were applied in 117 seconds. Then the elongation indexes occurred as a result were measured. At the second stage, 24 different stress models between 79,65-80,65 Pa were applied in 102 seconds in order to measure elongation index of erythrocyte under high stress conditions. At the third stage, following high stress model, 9 shear stress between 0,3-50 Pa were applied in 117 seconds. Deformability of erythrocytes was evaluated with elongation index values.According to our findings, elongation index values of HIS group before stress, were found to be low statistically significant between hemoglobin D and control group (p<0,05). Within stress model, elongation index values of HIS group before stress, were found to be low statistically significant between hemoglobin D and control group (p<0,05). Elongation index values of HIS group after stress, were found to be lower than those of hemoglobin D and control group significantly (p<0,05).It was detected that deformability of erythrocyte decreased in thalasemia intermedia, sickle cell anemia and hemoglobin H patient groups when compared with hemoglobin D group.Key Words: Erythrocyte deformability, hemoglobinopathy, hemoglobin D