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Old 10-04-2011
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Default Bruton agammaglobulinemia

A boy of 10 month of age presented with repeated sinopulmonary infections, on examination the you noticed diminished tonsils, adenoids & lymphnodes. Family history positive of uncle having the disease. Bruton agammaglobulinemia is highly considered in this case. What is the most characteristic finding that differentiate it from other immunodeficiency status?
  • Decrease in all immunoglobulins classes.
  • Diminished lymphoid organs.
  • Normal T cell mediated immunity.
  • Absent B cells in the peripheral blood.
  • Absent pre B cells in the marrow
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Old 10-04-2011
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Absent B cells in the peripheral blood.
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Old 10-04-2011
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Absent B cells in the peripheral blood
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Old 10-05-2011
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Correct answer: Absent B cells in the peripheral blood.
Remember for Bruton agammaglobulinemia: Male child (X-linked), deficiency of tyrosine kinase, blocks B cells maturation in BM at pre B stage, so no mature B cells, and then no Igs of all classes. The definitive test is flow cytometry. Symptoms start to develop after 6 month of age. Pyogenic infections, enteroviruses & hepatitis. Lymphoid hypoplasia is important feature. Treatment is IVIg monthly.
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