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

Multiple Choice

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:

A) Bruton's hypogammaglobulinemia
B) Wiskott-Aldrich syndrome
C) DiGeorge syndrome
D) ataxia telangiectasia