Brainscape's Knowledge GenomeTM

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


See full index

Mercy CLS 1: Immunology > 14) Immunodeficiency > Flashcards

14) Immunodeficiency Flashcards

1
Q

primary vs secondary immune deficiency syndromes

A

primary – deficiency is cause of disease

secondary – deficiency is a sx of disease

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

primary immunodeficiencies are categorized based on…

A

the expressed defect

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

SCID

A

severe combined immunodeficiency diseases

T and B cell defect

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

SCID patient suscpetible to…

A

any infection, especially…

  • CMV
  • Pneumocystis jiroveci
  • Candida
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

T- B+
(2 types)

A

X-linked SCID: T cells ↓ and B cells don’t function (lack of T cell ILs)

autosomal recessive SCID: same clinically; JAK3 mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

T- B-
(2 types)

A

adenosine deaminase deficiency: T/B cells don’t develop; skeletal abnormalities; toxic waste buildup

recombinase deficiency: Rag genes cannot rearrange; maturation stops at pre-T/B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Omenn syndrome

A

recombinase deficiency (SCID) with partial Rag activity

acts like graft-vs-host disease
infiltrates of T-cells, eos, and hyper-IgE in GI/skin

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

T+ B+
(3 types)

A

SCID with normal T/B numbers and abnormal function

  • bare lymphocyte syndrome
  • Wiskott-Aldrich syndrome
  • Ataxia telangiectasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

MHC is lacking; T-helpers do not get presented with Ag

A

bare lymphocyte syndrome

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

reversed CD4:CD8 ratio (more CD8)

thymic epithelials cannot select CD4+ cells

A

bare lymphocyte syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

triad of Wiskott-Aldrich syndrome

A
  • thrombocytopenia with small plts
  • recurrent bacterial infections
  • allergic rxn, hyper-IgE, food allergies, eczema
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

patients unable to respond to polysaccharide Ag

A

Wiskott-Aldrich syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

lifespan of 3 years without tx

cure is BM transplant

A

Wiskott-Aldrich syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

staggering gait + spiderlike vascular dilation

A

Ataxia telangiectasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

lymphopenia, thymic hypoplasia
↓ IgA, IgE, IgG
increased susceptiblity to malignancies

A

Ataxia telangiectasia

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

in T-cell immunodeficiencies, Ab may be lacking because…

A

lack of functional T-helpers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

patients with T-cell immunodeficiencies susceptible to…

A

viruses
bacteria
fungi
protozoa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

thymus and parathyroid do not form

↓Ca and lack of T-cells

no IgG (no class switching)

A

congenital thymic aplasia
(DiGeorge syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

cardiac disease
chronic infections

A

congenital thymic aplasia
(DiGeorge syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

selective defect in T-cells
no cell-mediated response to C. albicans

A

chronic mucocutaneous candidiasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

pre-B cells do not mature, so no Ig

severe, repeated bacterial infections when mom’s Ab is gone (5-6 months)

A

Bruton’s agammaglobulinemia (x-linked infantile agammaglobulinemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

repeated infections with…
H. influenzae
S. pneumoniae

A

Bruton’s agammaglobulinemia (x-linked infantile agammaglobulinemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Bruton’s agammaglobulinemia tx

A

IV IgG

24
Q

cannot make IgG from 2 months to 2 years

↓ T-cell number and function

A

transient hypogammaglobulinemia

25
Q

↓↓ IgG and IgA

peaks at 1-5yo and 15-20yo

A

common variable immunodeficiency disease (CVID)

26
Q

recurrent pyogenic infections in resp/GI tracts

increased autoimmune disease, cancer

A

common variable immunodeficiency disease (CVID)

27
Q

most common Ig deficiency and diseases

A

IgA

sinopulmonary infections, celiac disease

28
Q

severe infections with polysaccharide encapsulated organisms (H. influenzae, Pneumococcus)

A

IgM deficiency

29
Q

2 T/B interaction disorders

A
  • hyper-IgM syndrome
  • Duncan’s syndrome
30
Q

no class switching; ↑ IgM, ↓ IgG, IgA, IgE

boys 1-2 yo with recurrent resp infections

A

hyper-IgM syndrome

31
Q

CD40 ligand defect on T-cells

A

hyper-IgM syndrome

32
Q

T-cells not able to control B-cell proliferation during EBV infection

results in Burkitt’s lymphoma if pt survives

A

Duncan’s syndrome (x-linked lymphoproliferative disease)

33
Q

WBCs cannot interact with endothelium and enter tissues

A

leukocyte adhesion deficiency (LAD)

34
Q

integrin defect
no diapedesis
recurrent soft tissue infections

A

LAD I

35
Q

selectin ligand defect
WBC cannot bind to endothelials

A

LAD II

36
Q

pts do not show normal signs of infection (no pus)

A

LAD

37
Q

giant granules
abnormal function of plts, neuts, NK cells
neuro sx, skin hypopigmentation

A

Chediak-Higashi syndrome

38
Q

defect in respiratory burst
granulomas form

A

chronic granulomatous disease

39
Q

susceptible to S. aureus, Serratia, Aspergillus in first 1-2 years of life

A

chronic granulomatous disease

40
Q

monos cannot secrete TNF-𝛼
susceptible to Mycobacteria

A

interferon-𝛾 receptor deficiency

41
Q

associated with S. pneumo, S. pyogenes, H. influenzae, and SLE

A

C1, C4, C2 or C3 deficiency

42
Q

C5-C9 deficiency interferes with ——–, the primary defense against…

A

MAC
GN organisms, especially N. meningiditis

43
Q

lack C1 esterase inhibitor
too many anaphylatoxins
edema

A

hereditary angioedema

44
Q

proteins that normally inactivate C’ cannot bind to membrane

A

PNH

45
Q

3 common causes of secondary immunodeficiency

A
  • malnutrition
  • chemo
  • deliberate immunosuppression
46
Q

characteristics of tumor cells

A
  • stop in particular developmental stage
  • accumulate in large numbers
  • originate from single clone
47
Q

TdT+ in nucleus

A

B-ALL

48
Q

endemic in Africa
highly vacuolated lymphs

A

Burkitt’s lymphoma

49
Q

B-cells from lymph node follicles transform

A

follicular lymphoma

50
Q

BCL1 and BCL2

A

1: mantle cell lymphoma
2: follicular lymphoma

51
Q

lymphoma in MALT

associated with H. pylori, Sjogren’s syndrome, Hashimoto’s

A

marginal zone lymphoma

52
Q

free light chain deposits in kidney

A

amyloids

53
Q

overproduced IgM
hyperviscosity syndrome

A

Waldenstrom’s macroglobulinemia

54
Q

CD4+ and CD8+, but little or no CD3+

A

T-ALL

55
Q

T neoplasm of skin

A

Mycosis fungoides
leukemic phase: sezary syndrome

56
Q

HTLV-1

A

adult t-cell leukemia

57
Q

Hodgkin’s cells

A

Reed Sternberg

Mercy CLS 1: Immunology (19 decks)

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