Wiskott-Aldrich Syndrome

Question 1

What is the inheritance pattern for Wiskott-Aldrich Syndrome?

Answer 1

X-linked recessive

Question 2

What are the main clinical features of Wiskott-Aldrich Syndrome?

Answer 2

1. Thrombocytopenia
2. Atopic dermatitis
3. Recurrent infections (i.e. strep pneumonia, AOM, meningitis, sepsis)
4. P jiroveci, Herpes virus
5. EBV associated malignancies

Question 3

What are the main laboratory findings?

Answer 3

1. Impaired humoral immune response to polysaccharide antigens
2. Variable Ig, usu. low IgM, high IgA & IgE, slightly low IgG w/ normal IgG2
3. %T cells mod. reduced
4. Lymphocyte response to mitogens variable