Which combination of findings best supports a diagnosis of sickle cell disease?

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Multiple Choice

Which combination of findings best supports a diagnosis of sickle cell disease?

Sickle cell disease is caused by homozygous HbS, leading to chronic hemolytic anemia. The hallmark lab pattern is HbS as the dominant hemoglobin with little to no HbA on electrophoresis, and a strong reticulocytosis reflecting the bone marrow’s response to ongoing destruction of sickled red cells. That combination—severe anemia with reticulocytosis, HbS predominance, and HbA absent—best fits a diagnosis of sickle cell disease because it shows both the destruction of red cells and the molecular signature of HbS without HbA to counterbalance it. Seeing HbS about 40% with HbA around 60% points to sickle cell trait, where there is usually no significant hemolysis or anemia. A normal Hb with no abnormal cells, or macrocytosis with a normal retic count, does not explain the hemolysis and HbS expression seen in disease.

Sickle cell disease is caused by homozygous HbS, leading to chronic hemolytic anemia. The hallmark lab pattern is HbS as the dominant hemoglobin with little to no HbA on electrophoresis, and a strong reticulocytosis reflecting the bone marrow’s response to ongoing destruction of sickled red cells. That combination—severe anemia with reticulocytosis, HbS predominance, and HbA absent—best fits a diagnosis of sickle cell disease because it shows both the destruction of red cells and the molecular signature of HbS without HbA to counterbalance it.

Seeing HbS about 40% with HbA around 60% points to sickle cell trait, where there is usually no significant hemolysis or anemia. A normal Hb with no abnormal cells, or macrocytosis with a normal retic count, does not explain the hemolysis and HbS expression seen in disease.

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