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SICKLE CELL DISEASE: BACKGROUND AND MANAGEMENT

Wed ziyad alnajjar 1 , Saud Abdullah Alazdi 1 , Meshal Mohammed Alharbi 2 , Musaad Mohammed ALKHARISI 3 , Abdullah Abdulmohsen Abdullah Almulhim 4 , Sultan tariq fageeha5 , Abdulaziz Abdullah Abdulsattar Joharji 6 , Alqahtani Mohammed Saeed M 7 , ASHWAG MUSFER ALSHAHRANI 8 , AFAF Mohammad saad almugard 8

Introduction: Sickle cell disease is considered to be one of the commonest hereditary diseases around the world with being highly prevalent in the regions of sub-Saharan Africa, Mediterranean, Middle East, and Southeast Asia. Sickle cell disease is known to be a chronic hematological disease that leads to severe hemolysis, and is characterized by hemoglobin particles that tend to polymerize within the RBCs and cause the RBC to become in a crescent, sickle shape, which will cause hemolysis along with vasoocclusive events in different organs. Aim of the work: we tried to understand the pathogenesis, impact, diagnosis, and management of sickle cell disease in this study. Methodology: we conducted this review using a comprehensive search of MEDLINE, PubMed and EMBASE from January 1950 to March 2018. The following search terms were used: sickle cell anemia, hemoglobinopathy, genetics of sickle cell, complications of sickle cell, management of sickle cell disease Conclusion: It is important to develop therapeutic options that reduce the risk of complications of sickle cell disease. Despite all advances in treatment, sickle cell disease remains to be an important serious disease that is associated with severe morbidity and mortality, and poor quality of life. More research and clinical trials should be conducted on this disease to develop advanced treatment options that will further improve quality of life and decrease mortality and complications. Keywords: sickle cell disease, hemoglobinopathies, Middle-east hematological disorders.

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