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Flashcards in Immunodeficiency Diseases - Powell Deck (21)
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What is a Immunodeficiency?

Basically when, part of your immune system is missing or dysfunctional.


What are the 2 main types of immunodeficiencies?

Primary or congenital

Secondary or acquired immunodeficiencies


What is Primary immunodeficiency?

Are genetic defects that result in an increased susceptibility to infection.
Is frequently manifested infancy and childhood
*affects about 1 in 500 people


What is Secondary Immunodeficiency?

Develop as a consequence of:
- disseminated cancer
- Treatment with immune suppressive therapies
- infection of cells of the immune system.


Any loss of function mutation affecting a Toll-like receptor has what kind of effect?

Negative consequences for survival


________ disorders may affect one or more components of the immune system, including T & B lymphocytes and Nk cells.

Primary Immunodeficiencies


What is the Principal consequence of having an immunodeficiency?

Increased risk of infection


The types of _________ can predict the type of immunodeficiency.

type of recurring infection


Deficient _________ immunity usually results I'm increased susceptibility to infection by Pyogenic bacteria.



What are the defining features of XLA (Burton's Agammaglobulinemia)?

- Antibody isotypes are very low - not even IgM or IgD
- Circulating B cells are usually absent
- Pre B cells are reduced in #s in the bone marrow
- Tonsils are small and lymph nodes are barely palpable due to lack of germinal centers.
- Thymus structure is normal
- Most boys affected by XLA remain well during first 6-9 months of life (via maternal transmitted IgG)......Then infections start.


_______________ is associated with a loss of function of Bruton Tyrosine Kinase that is important for pre-B cell expansion and maturation into Ig-expressing B cells.



What are the characteristics of X-linked immunodeficiency with Hyper-IgM?

- Very low serum IgG, and IgE
- A elevation concentration of polyconal IgM
* Like XLA, patients may be asymptomatic during first year or two of life.
*In contrast to XLA, patients have Lymphoid hyperplasia.


__________ is associated with a loss of function of CD40 ligand that is expressed on helper T cells.

X-linked immunodeficiency with Hyper-IgM


What does the loss of CD40 ligand cause?

- Prevents the T cell from stimulating antigen-specify B cells.
- So B cells are not signaled buy T cells to undergo isotope switching and therefore only produce IgM.


What is the treatment for Immunodeficiency in Humoral Immunity?

Treatment is routine prophylactic antibiotics and/or gamma-globulin therapy.


Deficient ___________ immunity usually results in an increased susceptibility to viruses and other intracellular pathogens.

Cell-mediated immunity


What is the treatment for defects associated with Deficient T cell response?

Very few treatments
- rarely survive childhood.


What is DiGeorge's Syndrome?

This is a developmentally related disease associated with tissue morphogenesis - The Thymus does not develop!
*The # of T cells is very decreased and infants live months to 2 years.


What is X-linked SCID?

It is the most common "X-linked Recessive Severe Combined Immunodeficiency Disease.
*"Bubble Boy" severe combined immunodeficiency, fatal syndrome characterized by profound deficiencies of T & B cell function.


What are the 2 goals of treatment of Immunodeficiencies?

1. Minimize and control infections
2. Replace defective part or absent components (passive immunization or bone marrow transplant)


What is a prime example of a Secondary or acquired Immunodeficiency?