A 1-year-old boy is evaluated for a suspected immune deficiency.He has a history since birth of eczema,recurrent rashes,and diarrhea.(Normal values are given in parentheses.)His WBC count is 6.75/uL (5.0-10.8 × 10³ uL)with a normal differential.Platelet count is 35,000 (150,000-350,000/uL)and the platelets are small.His IgG is 750 mg/dL (600-1,500 mg/dL),IgM is 30 mg/dL (75-150 mg/d:),IgA is 475 mg/dL (50-125 mg/dL),and 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% (5-20%)CD19 and 50% (60-80%)CD3 cells with a normal ratio of CD4:CD8.T-cell function tests are abnormal.This boy most likely has:

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The boy's symptoms and laboratory findings are consistent with Wiskott-Aldrich syndrome, which is characterized by eczema, thrombocytopenia with small platelets, and immunodeficiency with abnormal antibody levels and T-cell function.

A 1-Year-Old Boy Is Evaluated for a Suspected Immune Deficiency