Combinations of sickle cell anemia and alpha thalassemia in Çukurova region

The hematological expression and pathological potential of hemoglobin S is influenced by several factors, including but not limited to, the presence of other abnormal intracellular hemoglobins or thalassemias. Alpha gene deletions, which are among the factors affecting this dynamic process, were the subject of our research due to their high prevalence in the Çukurova region. The association of α-thalassemia with sickle cell anemia trait (HbAS) results in a decrease in intraerythrocytic Hb S concentration, thus HbS polymerization, followed by less severe hemolytic anemia. However, the clinical significance of the interaction of α-thalassemia with sickle cell trait is controversial. Therefore, the aim of our study was to investigate the effect of α-thalassemia genotypes on clinical and hematological parameters in patients with sickle cell anemia trait. In this study, the hemogram findings of 98 cases aged 1-71 years who applied to Çukurova University Hospital for treatment were evaluated. According to the results of hemoglobin electrophoresis, it was observed that the Hb S ratio was below 45% in 90 of the 98 samples and was a carrier for the sickle cell gene. According to these results, DNA isolation was performed from the collected blood samples. Multiplex PCR method was used to determine alpha globin gene deletions. Fifty four cases with HbS trait were detected for 3 alpha gobin gen (3.7kb, 17.4kb ve 20.5kb) deletions. Keywords: HbS, Sickle cell anemia, Alpha thalassemia