Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Basic Pathology > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

immunodeficiency:

  • impaired _____
  • increased _____
  • often require _____
  • type of infection indicates _____
A 
immune function;
susceptibility to infections;
IV antibiotics (unusual and severe infections);
type of deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

immunodeficiency-associated infections:

T-cell deficiencies

A

viral, fungal, yeast, atypical microorganisms, cancer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

immunodeficiency-associated infections:

B-cell and phagocyte deficiencies

A

microorganisms requiring opsonization (largely bacterial infections)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

immunodeficiency-associated infections:

complement deficiencies

A

resemble B-cell deficiencies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

primary immunodeficiency

A
  • congenital
  • genetic anomaly
  • generally more severe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

secondary immunodeficiency

A
  • acquired
  • caused by another illness
  • more common
  • tend to be less severe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

primary immunodeficiencies:

  • ____ defect, mostly ____
  • manifest ____
  • between ____ in population
A

single gene;
sporadic;
early in life;
1:500 and 1:500,000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

primary immunodeficiencies:

five groups

A
  • B-lymphocyte deficiencies (most common)
  • T-lymphoctye deficiencies
  • phagocyte defects
  • combined T- and B-cell deficiencies
  • complement defects
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

B-lymphoctye deficiencies

A
  • hypogammaglobulinemia or agammaglobulinemia

- increased susceptibility to Ab-mediated infections (bacterial pneumonia, sinusitis, otitis media)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

causes of B-lymphoctye deficiencies

A
  • receptor defects: ineffective intracellular signaling (BCR and cytokine receptors)
  • Ab-class switch defect: altered ability to change Ab class (co-stimulatory signal defect)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

primary B-cell immundeficiencies

A
  • Bruton’s (X-linked) Agammaglobulinemia
  • Common Variable Immunodeficiency
  • Selective IgA Deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Bruton’s (X-linked) Agammaglobulinemia

A
  • most severe B-cell deficiency
  • receptor defect (BCRs never develop)
  • no mature B-cells
  • recurrent bacterial infections
  • unable to mount any humoral response
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Common Variable Immunodeficiency (CVID)

A
  • most common primary immune deficiency
  • 90% of cases are sporadic (no family hx)
  • class-switch defect: lacking IgG, IgA, and IgM
  • onset of symptoms is delayed until late 20s
  • recurrent bacterial respiratory infections
  • increased cancer risk
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Selective IgA Deficiency

A
  • unable to produce IgA Abs
  • compromised secretory immune system
  • severe allergies and recurrent sinus/lung/GI infections
  • severe mucopurulent conjunctivitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

primary T-cell immunodeficiencies

A

-DiGeorge Syndrome/22q11.2 Deletion Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

DiGeorge Syndrome/22q11.2 Deletion Syndrome

A
  • partial or complete absence of T-cell immunity (CD4+ and CD8+)
  • thymic aplasia or hypoplasia (little or no circulating T-cells)
  • endocrine, vascular, and facial abnormalities
  • viral, fungal, and intracellular bacterial infections
  • higher incidence of autoimmune disease
  • 90% of cases are sporadic
17
Q

Severe Combined Immunodeficiency (SCID)

A

-combined T- and B-cell deficiencies (some NK)

possible causes:

  • toxic metabolic by-products
  • cytokine receptor defects
  • V(D)J recombination defects
  • TCR defects: bare lymphocytes (class I: no Tc/CD8; class II: no Th/CD4)
18
Q

Reticular Dysgenesis

A
  • most severe SCID
  • absent/defective stem cell
  • no circulating lymphocytes or granulocytes
  • usually fatal before or soon after birth
19
Q

complement deficiencies

A
  • resemble B-lymphocyte deficiencies: bacterial infections
  • C3 deficiency: most severe
  • terminal complex deficiency (8000x increased risk of Neisseria gonorrhea infection)
20
Q

phagocytic deficiencies

A

-often resembles B-lymphocyte deficiencies: bacterial infections

types:

  • neutrophil maturation defect
  • adhesion molecule defect
  • opsonin receptor defect
  • pathogen killing defects

-chronic granulomatous disease

21
Q

chronic granulomatous disease

A
  • pathogen killing defect: absent oxygen-dependent mechanism

- recurrent granulomas in lung, skin, and bones

22
Q

secondary deficiencies:

  • ______ deficiencies
  • far more _____ than primary deficiencies
  • ____ prognosis
  • duration of _____
A

acquired;
common;
variable;
transient to chronic

23
Q

number one cause of secondary deficiencies

A

malnutrition

24
Q

HIV

A
  • RNA retrovirus
  • preferentially infects CD4+ Th1 cells
  • HIV also targets other cells in the body
25
Q
  • _____ HIV cases in US
  • _____ aren’t aware of their status
  • not distributed equally in population:
  • ______
  • ______
A

1.2 million;
1 in 7;
men who have sex with men are highest risk group;
AA > Latino > white

26
Q

HIV:

acute phase

A

mononucleosis-like syndrome 3 to 6 weeks after infection

27
Q

HIV:

clinical latency phase

A
  • asymptomatic period 2-10 years after infection (w/o Tx)

- CD4 T-cell count greater than 500 cells/mm3 of blood (normally between 500-1500 cells/mm3)

28
Q

HIV:

stage 3- AIDS

A
  • increasing risk of opportunistic infections

- CD4 T-cell count less than 200 cells/mm3 of blood

29
Q

HIV-related immunologic abnormalities

A
  • lymphopenia (low CD4+ T-cell count)
  • cutaneous anergy (defect in cell-mediated immunity)
  • hypergammaglobulinemia (polyclonal B-cell stimulation by Epstein-Barr virus)
  • CD4:CD8 ratio <1 (normally between 1 and 4)
30
Q

AIDS criteria

A
  • HIV +
  • CD4+ T-cell count < 200 cells/mm3
  • AIDS-defining condition
31
Q

most common AIDS-defining infections

A
  • pneumocystis jiroveci pneumonia

- systemic candidiasis

32
Q

evaluation of immunity

A
  • complete blood count (CBC) with a differential
  • quantitative Ig determination
  • assay for total complement
  • skin tests (tests T-cell immunity)
33
Q

treatment for immunodeficiencies

A
  • gamma-globulin therapy
  • transplantation or transfusion
  • treatment with soluble immune mediators
  • gene therapy
34
Q

gamma-globulin therapy

A
  • transient: 3-4 weeks
  • effective for B-cell deficiencies
  • relatively low risk
35
Q

transplantation or transfusion

A
  • bone marrow or umbilical cord blood-stem cells
  • effective for some primary combined deficiencies
  • higher risk of Graft vs Host Disease)

Basic Pathology (21 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions