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SP2017 Micro Exam 3 > Lecture 6- Immunodeficiency > Flashcards

Lecture 6- Immunodeficiency Flashcards

1
Q

Deficient…. usually results in increased susceptibility to infection by pyogenic bacteria

A

humoral immunity

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2
Q

Deficient…. usually results in increased susceptibility to viruses and other intracellular pathogens

A

cell mediated immunity

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3
Q

…. are genetic defects that increase susceptibility to infection and are manifested in infancy or early childhood

A

primary or congenital immunodeficiencies

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4
Q

…..develop as a consequence of malnutrition, disseminated cancer, tx with immunosuppressive drugs or infection of cells of the immune system

A

secondary or acquired immunodeficiencies

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5
Q

…. are conserved across widely diverse species

A

Toll- like receptors

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6
Q

Any loss of function mutation affecting a TLR has….

A

negative consequences for survival

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7
Q

Primary immunodeficiences may affect one or more components of the immune system, including…

A

T and B lymphocytes
NK cells
phagocytic cells
complemetn proteins

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8
Q

Immunodeficiencies may result from defects in ….. or from defects in effector mechanisms of innate and adaptive immunity

A

leukocyte maturation or activation

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9
Q

The principal consequence of an immunodeficiency is

A

an increased susceptibility to infection

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10
Q

In immunodeficiencies, the types of recurring infections can predict…

A

the type of immunodeficiency

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11
Q

In X-linked Agammaglobulinemia (XLA) all antibody isotypes are…. and circulating…. are usually absent

A
  • very low- not even IgM or IgD

- B cells

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12
Q

In XLA, Pre-B cells are present in …. in the…

A

reduced numbers

bone marrow

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13
Q

In XLA, tonsils are usually…. and lymph nodes are…. due to absence of….

A
  • very small
  • rarely palpable
  • germinal centers
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14
Q

In XLAM the thymus architecture is…. as are the…. areas of spleen and lymph nodes

A

normal

T cell dependent

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15
Q

Most boys afflicated wwith XLA, remain well during the first…. of life by virtue of maternally transmitted…. antibodies. Therefore, they repeatedly acquire infections with extracellular pyogenic organisms such as pneumococci, streptococci, and haemophilus

A

6-9 months

IgG

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16
Q

XLA is also known as….

A

Bruton’s agammaglobulinemia

17
Q

In XLA, … are usually absent and … are present in reduced numbers in the bone marrow

A

circulating B cells

Pre-B cells

18
Q

The defect in XLA is associated with a loss of function of…. that is important for Pre-B cell expansion and maturation into Ig-expressing B cells

A

Bruton Tyrosine Kinase

19
Q

Bruton Tyrosine Kinase is important for….

A

pre-B cell expansion and maturation into Ig-expressing B cells

20
Q

X-linked immunodeficiency with hyper-IgM is characterized by very low serum …,… and…

A

IgG, IgA, and IgE

21
Q

X-linked immunodeficiency with hyper-IgM is also characterized by a markedly elevated concentration of….

A

polyclonal IgM

22
Q

In contrast to patients with XLA, hyper-IgM patients have …..

A

lymphoid hyperplasia

23
Q

The defect in X-linked immunodeficiency with hyper-IgM is a loss of function of…. that is expressed on helper T cells

A

CD40 (aka CD154)

24
Q

The loss of CD40 (aka CD154) prevents the T cell from ….

Thus, B cells are not signaled by the T cell to go through…. and only produce…

A
  • co-stimulating antigen-specific B cells (through CD40)
  • isotype switching
  • IgM
25
Q

Treatment of humoral immunodeficiencies is….

A

routine– prophylactic antibiotics or gamma-globulin therapy

26
Q

There are few if any treatments for…

A

defects associated with deficient T cell responses so patients with absolute defects in T cell function rarely survive past infancy/childhood

27
Q

DiGeorge’s syndrome is a developmentally related disease associated with….

A

tissue morphogenesis– the thymus does not develop

28
Q

In digeorges syndrome, thymic hypoplasia results from defects in morphogenesis of the….

A

3rd and 4th pharyngeal pouches during early embryogenesis

29
Q

In digeorge’s syndrome, the percentage of T cells is variably…. as a result, there is a relative increase in the percentage of ….

A

decreased

increase

30
Q

In digeorge’s syndrome, B cell function is impaired only to the extent of needing..

A

T helper cells

31
Q

In digeorge’s syndrome, most infants die from…. within first few months or second year of life. Patients who survive infancy are usually…

A
  • seizures, cardiovascular defects, infections

- mentally retarded

32
Q

There are clinical similarites between digeorge’s syndrome and…

A

fetal alcohol syndrome

33
Q

Severe combined immmunodeficiency is a rare, fatal syndrome characterized by

A

profound deficiencies in T and B cell function

SP2017 Micro Exam 3 (8 decks)

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