Main menu


Case study (37) – Hemoglobin SC Disease

Case study (37) – Hemoglobin SC Disease 

A 22-year-old male complains of acute onset of generalized pains in the back, abdomen, and limbs. 

In the past week, his frequency and pain have also increased. 

Upon examination, his right upper arm was swollen and painful. 


Hemoglobin (Hb) 84 g/L 

Mean corpuscular value (MCV) 81 fL 

White blood cells (WBC) 33.4 X 109/L (neutrophils 86%) 

Platelets 390 V 109/L 


Q1. What abnormalities are shown in the below blood film? 

Q2. Hemoglobin electrophoresis was performed on the patient, his family, and controls: 

▪ Lane a is his mother. 

▪ Lane b is his father. 

▪ Lane c is his sister. 

▪ Lane d is his brother. 

▪ Lane e is the patient. 

▪ Lane f is another brother. 

▪ Lane g is a control sample of hemoglobin A/C blood. 

▪ Lane h is a control of hemoglobin A/S blood. 

Q3. What abnormality is on the x-ray, and what complication has occurred? 

Q4. What other skeletal complications may occur? 

Q5. What other renal complications may occur? 


A1. The blood film shows target cells and irregularly contracted red blood cells. 

Sickle cells are not prominent. Rectangular cells and cells in which the cytoplasm has shrunk away from the membrane are also seen. 

A2. The findings in the blood film and the hemoglobin electrophoresis patterns suggest hemoglobin SC disease, the compound heterozygous state for the two important beta-chain mutants, hemoglobin S and C. 

None of the siblings has a significant hemoglobinopathy, and none of them is likely to be symptomatic. 

SC disease is a variant of homozygous sickle cell anemia (SS disease) with almost the same severity and similar clinical manifestations. 

This patient appears to have a sickle cell crisis, which may precede a urinary tract infection. 

The figure below is an illustration of high-performance liquid chromatography (HPLC) in a sickle cell; this is now the standard diagnostic technique. 

A3. The x-ray shows sequestrum formation within the humerus. There is a periosteal reaction. This suggests osteomyelitis. 

Blood culture revealed bacteremia with Escherichia coli, and the same organism was isolated from the right humerus. 

Patients with sickle cell anemia are at increased risk of osteomyelitis because organisms may enter the blood from areas of mucosal damage (such as in the urinary tract and gastrointestinal tract), the spleen is weak, and the infarcted bone marrow area is easily colonized by organisms. 

Salmonella osteomyelitis is particularly common, because hyposplenism function is the cause of the organism, and bile produced by hemolysis in the bone marrow provides a rich medium. 

A4. The most important additional skeletal abnormality is avascular necrosis of the femoral head or the humeral head. 

A5. Renal papillary necrosis may occur in hemoglobin SC disease.