The sickle cell disease is fatal in nature. Thousands of children are dying off due to this health problem throughout the globe. Due to the rapid development of diagnosis and clinical managements such patients are living up to a respectable age. But as there is no permanent cure the patients are suffering from bone and joint pain, jaundice, hepato-splenomegaly, chronic infections etc. The main physiological complicacy is due to the polymerization of sickle hemoglobin (HbS), (sickling process) inside the red blood cell (RBC) of these patients during deoxygenating state. The change of RBC from spherical to sickle shape is due to the polymerization of mutant hemoglobin (HbS) inside the RBC and membrane distortion during anoxic condition. The mechanism and the process of sickling are very complex and multifactor in nature. To get rid from such complicacies it is necessary to suitably and accurately stop the sickling of RBC of the patients. The potential anti-sickling agents either from natural sources and/or synthetic molecules may be helpful for reducing the clinical morbidity of the patients. A lot of natural compounds from plant extracts have been tried by several workers in recent past. Most of the studies are based on in vitro red cell sickling studies and their mode of action has not been properly understood. Although, few studies have been in vivo in nature pertaining to transgenic sickle animal model, there is paucity of data on the human studies. The result of such studies although has shown some degree of success, a promising anti-sickling agent is yet to be established.

No abstract

Hemoglobinopathies are the cause of some major genetic and social health problem in many countries including India and sickle cell disorder is one of the most debilitating genetic disease affecting a large population. Though recognized, in and as various groups of genetic mutations, double heterozygous state of sickle cell and hemoglobin E (HbSE) is uncommon in Odisha state. Due to migration and racial inter-caste marriages between the populations of different neighboring states the possibilities of detecting Hb SE disease in Odisha is rising but this rare case of Hb SE has never been reported in Balasore district so far. In the present instance, a 24 year old male was diagnosed with sickle cell hemoglobin E disorder. The case was confirmed through Hb electrophoresis, slide based sickle test, HPLC and ARMS PCR. The peripheral smear analysis showed the presence of microcytic RBC and Hypochromasia. No splenomegaly and hepatomegaly was observed. The case was mostly asymptomatic and with history of joint and bone pains once or twice per year.