Introduction: Haemoglobinopathies are the most common inherited red cell disorders worldwide. Identification of these disorders is immensely important epidemiologically, for early diagnosis, improved management protocols and prevention of disease in upcoming generation. Our aim is to study the abnormal haemoglobin variants in patients of anaemia using High Performance Liquid Chromatography (HPLC) BIO-RAD D-10 Dual Program analyser in Vadodara region of Gujarat. Methods: A total of 1890 cases of patient's with anaemia having haemoglobin up to 11 gm% were studied for HPLC for diagnosing any abnormal haemoglobin disorder by BIO-RAD D-10 Dual Program analyser. Results: Out of 1890 cases of anaemia studied, 1236 (65.3%) cases showed abnormal haemoglobin, and rest 654 (52.9%) cases showed normal result on HPLC. Of these, 1236 cases, 686 cases of sickle cell trait (55.5%) the predominant abnormality, followed by 247 cases of sickle cell disease (19.9%). There were 208 cases of beta Thalassemia trait (16.8%), followed by 89 cases of double heterozygous state of beta thalassemia and sickle cell trait (7.2%). There were three cases of Thalassemia major with high Hb F (0.24%), two cases of Hb D Punjab heterozygous patients (0.16%) and one case of Hb E heterozygous (0.08%). Conclusion: HPLC is an accurate, simple and superior technique in detection of various haemoglobin disorders, which helps in management of patients and prognostic significance. It is also important in premarital and antenatal screening tests, to prevent birth of children with severe Haemoglobin disorders.
Introduction:The individuals having thalassemia trait usually have an asymptomatic course and have mild microcytic hypochromic anemia. Since the other cause of microcytic anemia is iron deficiency, it is important to differentiate it from thalassemia trait. Though the definitive diagnosis of thalassemia trait is possible only by HPLC, the Mentzer index is such an index that can differentiate between thalassemia trait and iron deficiency anemia. Objectives: This study aims at figuring out the diagnostic value and reliability of the Mentzer index in the differentiation of iron deficiency anemia and thalassemia carrier compared with the results of HPLC. Methods: This was a hospital-based retrospective observational study done on 1236 patients of all age groups. Mentzer index of all the patients was calculated and the results were analyzed and further compared with HPLC. Results: Out of 1236 patients, 741 (59.9%) patients are of iron deficiency anemia and 495 (40.1%) patients are of β thalassemia trait. Mentzer index was found to be more reliable to detect true positive cases for β thalassemia trait with a sensitivity of 89.0%, specificity of 87.9%. Positive predictive value for β thalassemia trait is 83.2% and Negative predictive value is 92.3% with Youden's Index 76.9. Conclusion: Iron deficiency anemia and thalassemia have different effects on blood indices. In resource-poor and developing countries like that of India, it can be used as a screening tool. In doubtful cases, the diagnosis can be confirmed by HPLC.
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.
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