Biological particularities of sickle cell syndrome

Abstract

Introduction: Sickle Cell Syndrome is a real health problem because of its frequency and chronic evolution. In the present work, we propose to study the biological particularities of a population of 66 patients. Patients and methods: This is a descriptive and cross-sectional prospective study carried out on a population of 66 patients with major sickle cell disease in the steady state (36 S/S, 18 S/β- thalassémics, 7 S/C and 5 S/OArab) over a two-year period (January 2018-December 2019) followed at the outpatient haemoglobinopathy clinic of the children's hospital in Tunis. Each patient was given haematological and biochemical test. Results: The population studied is composed of 66 patients with sickle cell syndrome with an average age of 15.5 ± 8.4 years. The anemia is normocytic normochromic in S/S, S/OArab and S/C. On the other hand it is microcytic hypochromic in S/β-thalassemics. Hyperleukocytosis and thrombocytosis were found in all but not in S/C patients. The rate of HbF is relatively high in our population. Hemolysis has been reported in all phenotypes but moderately in S/C patients with increases in total and direct bilirubin, LDH activity and AST activity which are more marked in S/S patients. Conclusion: Our study concluded that the biological data are different from one type of syndrome to another; the S/C phenotype being the best tolerated form.