Intrinsic hemolytic normocytic anemia Quiz: Ace Your Exams

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A 6-year-old girl is brought to the clinic by her parent due to repeated episodes of jaundice, pallor, and fatigue for the last 8 months and new-onset abdominal pain for the last 3 days. She is at the 35th percentile for height and 20th percentile for weight. The parent reports no pregnancy or birth-related complications and states that the patient had been relatively healthy previously. The parent states that the girl’s biological father had been hospitalized with similar symptoms as a child and had abdominal surgery, but the details of the surgery are unknown. The patient’s temperature is 37°C (98.9°F), pulse is 90/min, respirations are 24/min, and blood pressure is 118/72 mmHg. Physical examination shows an ill-appearing pale child with scleral icterus and splenomegaly. Laboratory tests are obtained and the results are shown below.  
 
 Laboratory value  Result  Reference Range 
 Hematologic   
 Leukocyte count   6,500/mm3  4,500-11,000/mm3  
 Platelet count  250,000/mm3   150,000-400,000/mm3  
 Hemoglobin  9.6 g/dL  12-16 g/dL 
 Mean corpuscular volume (MCV)   84 fL  80-100 fL 
 Mean corpuscular hemoglobin concentration (MCHC)  42 g/dL  25.4-35.6 g/dL 
 Red cell distribution width (RDW)  15%  11.8-14.5% 
 Reticulocyte count   9.1%  0.5-1.5% 
   
The patient most likely has a defect in which of the following? 

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