American Board of Internal Medicine (ABIM) Certification Practice Exam

In patients with sickle cell disease, which hemoglobin levels can typically be expected in Hb SS?

1. 5 to 6 g/dL

2. 6 to 7 g/dL

3. 7 to 8 g/dL

4. 8 to 9 g/dL

The correct answer is: 6 to 7 g/dL

In patients with sickle cell disease, specifically those with Hb SS genotype, the expected hemoglobin levels generally fall within the range of 6 to 7 g/dL. This is reflective of the chronic hemolytic anemia that characterizes this condition. In sickle cell disease, the sickling of red blood cells leads to their early destruction, resulting in lower hemoglobin levels compared to the normal range. This range of hemoglobin (6 to 7 g/dL) is significant as it indicates the degree of anemia typically observed in these patients. Patients often experience a variety of related symptoms due to decreased oxygen-carrying capacity, which contributes to the management and treatment strategies for sickle cell disease. While hemoglobin levels can fluctuate based on factors like hydration status, infections, and pain episodes, the chronic baseline is commonly within this range for those with Hb SS. Understanding these levels is essential for clinicians in diagnosing and monitoring the condition and in making informed decisions regarding treatment interventions and potential complications such as vaso-occlusive crises. Hemoglobin levels lower than this range indicate more severe anemia, which may arise from complications or inadequate management of the disease.