X-linked Agammaglobulinemia and Common Variable Immunodeficiency
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Questions and Answers

What is the main reason behind the lack of B-cells in X-linked agammaglobulinemia (Bruton’s)?

  • Deficiency in the production of antibodies
  • Excessive production of light chains inhibiting B-cell development
  • Overproduction of T-cells leading to suppression of B-cells
  • Inability of pro-B cells to differentiate into pre-B cells due to tyrosine kinase deficiency (correct)

Which condition is characterized by hypogammaglobulinemia, reduced lymphocytes, and decreased T-cell activity?

  • Common variable immunodeficiency (correct)
  • Isolated IgA deficiency
  • X-linked agammaglobulinemia (Bruton’s)
  • Hypogammaglobulinemia

What is a common symptom associated with Isolated IgA deficiency?

  • Otitis media (correct)
  • Arthritis
  • Kidney failure
  • Heart disease

Why can a celiac test result show a false negative in a patient with IgA deficiency?

<p>Low IgA levels in blood serum affect the test accuracy (C)</p> Signup and view all the answers

What is the primary biological defect in Hyper IgM syndrome?

<p>Deficiency or lack of function of CD40L (D)</p> Signup and view all the answers

Which immune deficiency can result in life-threatening anaphylaxis post blood transfusion due to recognition of transfused IgA as foreign?

<p>Isolated IgA deficiency (A)</p> Signup and view all the answers

Which of the following is true about hyper IgM syndrome?

<p>It is an autosomal recessive disorder that primarily affects males (A)</p> Signup and view all the answers

What is the prognosis for hyper IgM syndrome?

<p>Poor prognosis (C)</p> Signup and view all the answers

DiGeorge syndrome is also known as:

<p>22q11 deletion syndrome (D)</p> Signup and view all the answers

What is the primary biological cause of DiGeorge syndrome?

<p>Abnormal development of the 3rd and 4th pharyngeal pouches, leading to a small thymus and T-cell deficiency (C)</p> Signup and view all the answers

What is the primary immunodeficiency associated with severe combined immunodeficiency (SCID)?

<p>Mutation in cytokine receptors or the RAG gene, leading to a lack of T lymphocytes and NK cells (B)</p> Signup and view all the answers

Which of the following is an example of an innate immunodeficiency?

<p>Hereditary angioedema (D)</p> Signup and view all the answers

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