Wiskott-Aldrich Syndrome Flashcards

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1
Q

What is the inheritance pattern for Wiskott-Aldrich Syndrome?

A

X-linked recessive

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2
Q

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

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

What are the main laboratory findings?

A
  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
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