DOI: 10.36347/sjams.2018.v06i06.003

Pages: 2316-2332

Haemoglobinopathies are the common genetic disorders of haemoglobin.Identification of these disorders are immensely important epidemiologically. HPLC-The bio-rad variant-II haemoglobin testing system is a totally automated Ce-Hplc–(Cation Exchange High Performance Liquid Chromatography) instrument for routine identification and quantification of normal and abnormal haemoglobin. To monitors and understands the natural history of haemoglobinopathies in southern Odisha in paediatric age group. To report the cases of rare variants of haemoglobin found in the population of Southern Districts of Odisha. To assess the accuracy and precision of diagnosis of haemoglobinopathies by HPLC and its possible advantage over conventional techniques. A total of 435 blood samples of MICROCYTIC HYPOCHROMIC ANAEMIA cases were analysed referred from different peripheral hospitals of SOUTHERN part of Odisha state for a period of two years (2015-2017). 2 ml of iv blood samples were collected after obtaining informed consent from each individual. Haematological indices were measured using automated cell counter (SYSMEX-Xt 2000i) and HPLC (BIO-RAD VARIANT-II). Background data of each individual were recorded like age, sex ,caste, place of origin and consanguinity etc.Haemoglobin electrophoresis were carried out on agarose gel in Tris-EDTA-borate buffer at PH-8.9 and sickling test were carried out .Hb electrophoresis in acidic medium PH-6.2 was also carried out to confirm the presence of HbD or HbE band. Family studies were carried out for confirming the disease. Out of 435 cases 257 cases came to be positive. Sickle cell disease(23.2%), Sickle cell trait(17.9%), B-thalassemia trait(6.8%), Sickle cell-b-thalassemia(5.2%), Beta-thalassemia major(2.9%), HPFH (0.9%), Alpha-thalessemia-(0.6%), B-thalassemia intermedia-(0.6%), Lepore-heterozygote-(0.2%), Delta-b thalassemia-(0.2.%), E-B-thalassemia-(0.2%), The paediatric population is harbouring almost all major