Abstract
Introduction: Sickle cell anemia (SCA) is a genetic disorder characterized by severe hemolytic anemia. Diagnosis is an important aspect of the management of the disease. In the present study, the efficacy of the solubility test was assessed and High-performance liquid chromatography (HPLC) in the diagnosis of Sickle cell disorders. Material and Methods: A total of 1890 patients were screened. Among them, 1022 samples were tested positive for Sickle cell disorders. All the samples were observed for peripheral smear, subjected to solubility test and were further analyzed on HPLC ‘BIO-RAD’ analyzer for the confirmation along with the distinction of Sickle cell trait (SCT), sickle cell disease (homozygous) and Heterozygous sickle cell and beta-thalassemia. Results: Out of all 1890 patients screened, 1022 samples were found to be positive with the solubility test. A total of 686 samples were diagnosed as Sickle cell Trait, 247 for sickle cell disease (homozygous) and 89 samples were diagnosed as Sickle cell beta-thalassemia (heterozygous) with HPLC. Conclusion: Solubility test is very effective with 96.8% sensitivity. Specificity calibrated was on average 29.6%, with the predictive value of positive test 87.9% and a predictive value of negative test 64.0%. The most commonly used method now in Gujarat is HPLC although the solubility test is also sensitive and cheap.
Highlights
Sickle cell anemia (SCA) is a genetic disorder characterized by severe hemolytic anemia
A total of 686 samples were diagnosed as Sickle cell Trait, 247 for sickle cell disease and 89 samples were diagnosed as Sickle cell beta-thalassemia with High-Performance Liquid Chromatography (HPLC)
A total of 686 samples were diagnosed as Sickle cell Trait, 247 for sickle cell disease and 89 samples were diagnosed as Sickle cell betathalassemia with HPLC (Table 5)
Summary
Sickle cell anemia (SCA) is a genetic disorder characterized by severe hemolytic anemia. 1022 samples were tested positive for Sickle cell disorders. All the samples were observed for peripheral smear, subjected to solubility test and were further analyzed on HPLC ‘BIO-RAD’ analyzer for the confirmation along with the distinction of Sickle cell trait (SCT), sickle cell disease (homozygous) and Heterozygous sickle cell and beta-thalassemia. Results: Out of all 1890 patients screened, 1022 samples were found to be positive with the solubility test. Sickle cell disease is a hereditary blood disorder due to defective hemoglobin structure [1]. The disease results from a single base Adenine>Thiamine mutation in the triplet encoding the sixth residue of the β-globin chain, leading to a substitution of valine for glutamic acid and the abnormal hemoglobin S (HbS)
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