<unk>  is a group of genetic disorders of the red blood<unk>  caused by a mutation in the β-globin chain gene of hemoglobin at the 6th position replacing glutamic acid to valine. HbS polymerizes reversibly when deoxygenated to form a network of fibrous hemoglobin polymers that stiffens the RBC membrane, giving it a<unk>  shape. These<unk> d<unk> s loose the pliability to cross thin capillaries and possess a sticky membrane, giving it a property to adhere to the endothelium of blood vessels, thereby causing vaso-occlusion. It causes significant morbidity and mortality, particularly in people in the Mediterranean and African region. The treatment includes measures to avoid vaso-occlusion, such as avoidance of dehydration, low oxygen conditions, or cold weather. Hydroxyurea has been used to increase fetal hemoglobin production.
Sickle-cell disease
Alkaptonuria
Thalassemia
Argininemia
Hemoglobinopathy
Hereditary spherocytosis
Cryoglobulinemia
Trimethylaminuria