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A 1-year-old boy is evaluated for a suspected immune deficie…
A 1-yeаr-оld bоy is evаluаted fоr a suspected immune deficiency. He has a history since birth of eczema, recurrent rashes, and diarrhea. His white blood cell (WBC) count is 6.75/uL (normal values: 5.0–10.8 × 103 uL) with a normal differential. His platelet count is 35,000 (normal values: 150,000–350,000/uL), and the platelets are small. His IgG is 750 mg/dL (normal values: 600–1,500 mg/dL); IgM is 30 mg/dL (normal values; 75–150 mg/dL); IgA is 475 mg/dL (normal values: 50–125 mg/dL); IgE is 750 mg/dL (10–50 mg/dL). The boy's blood is typed as O-positive, and he does not have anti-A or anti-B antibodies. Flow cytometry performed on his blood shows 11% CD19 (normal values: 5%–20%) and 50% CD3 cells (normal values: 60%–80%) with a normal ratio of CD4:CD8. T-cell function tests are abnormal. This boy most likely has:
A 1-yeаr-оld bоy is evаluаted fоr a suspected immune deficiency. He has a history since birth of eczema, recurrent rashes, and diarrhea. His white blood cell (WBC) count is 6.75/uL (normal values: 5.0–10.8 × 103 uL) with a normal differential. His platelet count is 35,000 (normal values: 150,000–350,000/uL), and the platelets are small. His IgG is 750 mg/dL (normal values: 600–1,500 mg/dL); IgM is 30 mg/dL (normal values; 75–150 mg/dL); IgA is 475 mg/dL (normal values: 50–125 mg/dL); IgE is 750 mg/dL (10–50 mg/dL). The boy's blood is typed as O-positive, and he does not have anti-A or anti-B antibodies. Flow cytometry performed on his blood shows 11% CD19 (normal values: 5%–20%) and 50% CD3 cells (normal values: 60%–80%) with a normal ratio of CD4:CD8. T-cell function tests are abnormal. This boy most likely has:
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