"Haemoglobin SC (HbSC) disease, although a sickle cell disease subtype, with similarities to the classic condition, should ideally be considered as a distinct pathological entity 7."
"Individuals with one HbS beta chain and one haemoglobin C (HbC) beta chain, have a subtype of sickle cell disease known as haemoglobin SC (HbSC) disease 7."
Expected headings
"Skeletal"
"Pulmonary"
"Cardiovascular"
"Abdominal"
"Cerebral"
"Extramedullary haematopoiesis"
"Sickle cell disease (SCD) (historically also known as drepanocytosis) is a hereditary (autosomal recessive) condition resulting in the formation of abnormal haemoglobin (a haemoglobinopathy), which manifests as multisystem ischaemia and infarction, as well as haemolytic anaemia."
"There is no recognised gender predilection. The highest incidence occurs in individuals of African descent, followed by eastern Mediterranean and Middle Eastern populations. Malaria is the strongest known selective pressure on the human genome. The sickle cell mutation is prevalent in part as it confers a human genetic resistance to malaria. As the abnormal haemoglobin has higher turnover and increased rate of phagocytosis while sickled, red cells have reduced cell-cell cytoadherence preventing the parasite from multiplying during the erythrocytic phase of its life cycle. It is estimated that approximately 8% of the African population is homozygous for sickle cell (where malaria is most prevalent)."