A 1-year-old boy is evaluated for a suspected immune deficiency. He has a history since birth of eczema, recurrent rashes, and diarrhea. His white blood cell count (WBC) count is 6.75/uL (normal values: 5.0-10.8 x 103 uL) with a normal differential. 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. Bruton's tyrosine kinase deficiency.
B. Wiskott-Aldrich syndrome.
C. DiGeorge anomaly.
D. ataxia telangiectasia.


Ans: B. Wiskott-Aldrich syndrome.

Biology & Microbiology

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