Bare Lymphocyte Syndrome
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Questions and Answers

Which of the following is a disease caused by a failure to express class II major histocompatibility complex (MHC) molecules?

  • Hemophagocytic lymphohistiocytosis (HLH)
  • Th1 responses
  • Bare lymphocyte syndrome (correct)
  • Selective T cell deficiency
  • Which of the following is true about the bare lymphocyte syndrome?

  • It is associated with nontuberculous mycobacterial infections.
  • It affects the differentiation of naive T cells into effector cells.
  • It leads to a decrease in CD4+ T cells due to defective maturation in the thymus. (correct)
  • It is caused by mutations affecting various signaling pathways or cytokines.
  • Which of the following is a characteristic of hemophagocytic lymphohistiocytosis (HLH) syndromes?

  • It is associated with fungal and bacterial infections.
  • It is a disease caused by a failure to express class II major histocompatibility complex (MHC) molecules.
  • It is caused by mutations affecting various signaling pathways or cytokines.
  • It is characterized by systemic activation of immune cells, usually in response to infections. (correct)
  • Which type of mutations can result in persistent infections and excessive production of IFNγ?

    <p>Mutations in genes encoding proteins involved in granule exocytosis</p> Signup and view all the answers

    What is the main consequence of excessive macrophage activation in patients with these mutations?

    <p>Ingestion of red blood cells by highly activated macrophages</p> Signup and view all the answers

    What is the syndrome called in patients with mutations resulting in persistent infections and excessive macrophage activation?

    <p>Red blood cell ingestion syndrome</p> Signup and view all the answers

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