Bare Lymphocyte Syndrome

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

Bare lymphocyte syndrome

Which of the following is true about the bare lymphocyte syndrome?

It leads to a decrease in CD4+ T cells due to defective maturation in the thymus.

Which of the following is a characteristic of hemophagocytic lymphohistiocytosis (HLH) syndromes?

It is characterized by systemic activation of immune cells, usually in response to infections.

Which type of mutations can result in persistent infections and excessive production of IFNγ?

Mutations in genes encoding proteins involved in granule exocytosis

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

Ingestion of red blood cells by highly activated macrophages

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

Red blood cell ingestion syndrome

Test your knowledge on the bare lymphocyte syndrome and its impact on T cell activation. Learn about the mutations in transcription factors that lead to the failure of class II major histocompatibility complex (MHC) molecule expression. Explore how this affects the presentation of peptide antigens for recognition by CD4+ T cells.

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