Immunodeficiency Flashcards
What is immunodeficiency?
A failure of the immune response
What is the difference between primary and secondary immunodeficiency?
Primary is congenital and usually the result of a genetic defect
Secondary is acquired throughout life and is the result of another condition
Is primary immunodeficiency usually a result of a dominant or recessive gene and why is this the case?
Recessive - especially if the condition is more serious otherwise people with very severe immunodeficiency would not live to be able to reproduce
How do B cell deficiencies present?
Pyogenic bacterial infections and reduced IgG levels
What do histopathologic sample of B cell deficiencies show in a post mortem?
Reduced follicles and germinal centres in lymphoid organs
How do T cell deficiencies present?
Viral, fungal and protozoal infections and viral associated malignancies
Which class of immunodeficiency is most common?
B cell deficiency
Bone marrow transplants can be used as treatment for what class(es) if immunodeficiency?
T cell deficiency
Combined t and b cell deficiency
What is the treatment for a phagocyte deficiency?
Antibiotics
Cytokines
What is the treatment for b cell deficiencies?
Immunoglobulin injections
What is the treatment for complement deficiencies?
Infusions of complement components
What type of infections do phagocyte deficiencies present with?
Bacterial infections
What is the presentation of t an b cell combined immunodeficiencies?
Bacterial, viral, fungal and protozoal infections
What is the presentation of complement immunodeficiency?
Bacterial infections and autoimmunity
Describe the pathophysiology of bruton’s aggamaglobulinaemia
There is a mutation in the bruton’s tyrosine kinase gene for the receptor which responds to signals which allow the development of pre b cells to an immature b cell and prevents b cell development leading to low serum antibody levels
