Management of acute complications of sickle cell disease

Abstract

Sickle cell anemia (SCA) is an autosomal recessive disease characterized by the presence of hemoglobin S. It presents with hemolytic anemia e vaso-occlusives phenomena. The term sickle cell disease (SCD) includes the SCA and a group of compound heterozygous states in which the person has only one copy of the mutation that causes HbS and one copy of another abnormal haemoglobin allele, like sicklehemoglobin C disease (HbSC), sickle beta-thalassaemia and other rarer combinations. There are many acute complications of SCD: vaso-occlusives crisis, encapsulated organisms infections, mainly of the airways and sepsis, acute chest syndrome, splenic sequestration, priapism, stroke and aplastic crisis.The knowledge of these complications is very important for all grades of medical assistence. The early detection allows appropriate treatment and reduction of the morbidity and mortality associated with the disease.