Which of the following is a sickle cell condition?

• A. Sickle Cell anaemia
• B. Sickle cell trait
• C. HbSC disease
• D. HbS beta-thalassemia

Answer: (A)

Sickle cell disease centers on red blood cells that contain abnormal hemoglobin S, which sticks together when oxygen levels drop and makes cells become rigid and sickle-shaped. This leads to ongoing hemolysis and painful, sometimes organ-damaging crises. The clearest example of a sickle cell condition is sickle cell anaemia, which happens when a person inherits the HbS mutation from both parents (two HbS genes, HbSS). That genetic setup produces the classic, symptomatic disease with chronic anemia and vaso-occlusive complications. Sickle cell trait, by contrast, is a carrier state with one HbS allele and one normal HbA allele and is usually without symptoms. HbSC disease and HbS beta-thalassemia are other forms of sickle cell disease arising from different combinations of mutations, and they can cause disease too, but the classic, textbook sickle cell condition described here is sickle cell anaemia.