Immunodeficiency- powell Flashcards
Primary or congenital immunodeficiencies
are genetic defects that result in an increased susceptibility to infection
- manifest in early childhood
- effect 1 in 500 people
Secondary or acquired immunodeficiencies
develop as a consequence of
- malnutrition,
- disseminated cancer,
- treatment with immunosuppressive drugs, or
- infection of cells of the immune system.
Deficient humoral immunity usually results in what?
increased susceptibility to infection by pyogenic bacteria.
X-linked Agammaglobulinemia is also known as what?
Bruton’s Agammaglobulinemia
What are the symptoms of X-linked Agammaglobulinemia?
All Antibody Isotypes are very low – not even IgM or IgD
Circulating B cells are usually absent.
Pre-B cells are present in reduced numbers in the bone marrow.
Tonsils are usually very small and lymph nodes are rarely palpable due to absence of germinal centers.
X-linked immunodeficiency with hyper-IgM is characterized by what?
- very low serum IgG, IgA, and IgE
- a markedly elevated concentration of polyclonal IgM
what is the main difference between XLA and Hyper-IgM?
In contrast to patients with XLA, hyper-IgM patients have lymphoid hyperplasia
how does Hyper-IgM effect B cell development?
B cells are not signaled by the T cell to go through isotype switching and only produce IgM.
what is the treatment for deficient humoral immunity?
Treatment is routine – prophylactic antibiotics and/or gamma-globulin therapy.
what is the result of Deficient cell-mediated immunity
esults in increased susceptibility to viruses and other intracellular pathogens.
what is DiGeorge’s syndrome?
developmentally-related disease associated with tissue morphogenesis – the thymus does not develop.
what is the most common X-linked recessive immunodeficiency disease?
X-linked SCID (XSCID) is the most common form, accounting for approximately 42% of cases
what do XSCID patients have low levels of?
few or no T cells, and NK cells
have ELEVATED B cells
what are the treatments for immunodeficiencies?
- to minimize and control infections and 2. to replace the defective or absent components of the immune system
