Vol. 9, Issue 8 (2020)

Author(s):
Madhuri D Shende

Abstract:
Sickle cell disease (SCD) is a genetic disorder that affects erythrocytes (RBCs) causing them to become sickle or crescent shaped. The effects of this condition due to an abnormality of the hemoglobin molecules found in erythrocytes. The substitution of one amino acid in the hemoglobin molecule results in sickle hemoglobin. As a result RBCs sickle in low oxygen states causing occlusion of blood vessels, increased viscosity and inflammation. These RBCs are prematurely removed from the circulation, resulting in a chronic hemolytic anemia. With new born screening and early treatment, the death rate among children with SCD has declined. In addition, a variety of treatments are being introduced to help manage the various manifestations of disease. Transfusion, simple or exchange, is a mainstay of therapy, since it reduces the amount of Hgb S in circulation and suppresses erythropoiesis. Transfusion is indicated for symptomatic anemia and specifically to prevent stroke (first or recurrent), during acute stroke, and for acute chest syndrome. Unfortunately, transfusion carries risks for infectious disease transmission, as well as immunologic and inflammatory sequelae. For patients with SCD who may be chronically transfused, iron overload occurs frequently. In addition, due to differences in RBC antigens between donors and recipients, these patients are at increased risk for development of RBC alloantibodies, which can complicate further transfusion. It is, therefore, important to prevent all immunization by transfusing leukoreduced RBCs that match the patient for the C, E, and K1 antigens. Human progenitor cell (from bone marrow, peripheral blood stem cells, or umbilical blood) transplant can cure the disease for whom conventional therapy may not be effective. The care of people with sickle cell disease may include infection prevention with vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication. Other measures may include blood transfusion and the medication hydroxycarbamide (hydroxyurea). A small percentage of people can be cured by a transplant of bone marrow cells.