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Immunodeficiency Flashcards

1
Q

Which bacterial infections are assoc w/ No T Cells?

A

Sepsis

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2
Q

Which bacterial infections are assoc w/ No B Cells?

A

Encapsulated bacteria

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3
Q

What are the encapsulated bacteria?

A
  • S. pneumoniae
  • H. flu Type B
  • N. meningitidis
  • Salmonella
  • Klebsiella pneumonia
  • Group B Strep
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4
Q

Which bacterial infections are assoc w/ No Granulocytes?

A
  • Staph
  • Burkholderia cepacia
  • Serratia
  • Nocardia
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5
Q

Which bacterial infections are assoc w/ No Complement?

A

Neisseria (no MAC)

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6
Q

Which viral infections are assoc w/ No T cells?

A
  • CMV
  • EBV
  • VZV
  • Chronic infection w/ Resp/GI virus
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7
Q

Which Viral infections are assoc w/ No B cells?

A
  • Enteroviral encephalitis
  • Poliovirus (live vaccine CI)
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8
Q

Which viral infections are assoc w/ No granulocytes?

A

N/A

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9
Q

Which viral infections are assoc w/ No Complement?

A

N/A

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10
Q

Which Fungi/parasite infections are assoc w/ No T cells?

A

Candida & PCP

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11
Q

Which Fungi/parasite infections are assoc w/ No B cells?

A

GI giardiasis (no IgA)

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12
Q

Which Fungi/parasite infections are assoc w/ No Granulocytes?

A

Candida & Asperigillus

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13
Q

Which Fungi/parasite infections are assoc w/ No complement?

A

N/A

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14
Q

What do B-cell deficiences tend to produce?

A

Recurrent bacterial infections

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15
Q

What do T-cell deficiences tend to produce?

A

More fungal & viral infections

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16
Q

What are the B-cell deficiency disorders?

A
  • X-linked (Bruton’s) agammaglobulinemia
  • Selective IgA deficiency
  • Common variable immunodeficiency
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17
Q

What is the defect in X-linked (Bruton’s) agammaglobulinemia?

A

Defect in BTK, a tyrosine kinase gene→ no B cell maturation

X-linked recessive

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18
Q

How do X-linked (Burton’s) agammaglobulinemia pts present?

A

Recurrent bacterial infections after 6mo (dec maternal IgG) as a result of opsonization defect

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19
Q

What are the findings in X-linked (Bruton’s) agammaglobulinemia?

A
  • Normal pro-B
  • Dec maturation
  • Dec # of B cells
  • Dec Ig of all classes
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20
Q

What is the defect in Selective IgA deficiency?

A

Unknown

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21
Q

What is the MC primary immunodeficiency?

A

Selective IgA deficiency

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22
Q

How do pts present w/ Selective IgA deficiency?

A
  • Majority asx
  • Sinopulmonary infections
  • GI infections
  • Autoimmune dz
  • Anaphylaxis to IgA-containing blood products
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23
Q

What are the findings of Selective IgA deficiency?

A
  • IgA ,7mg/dL w/ normal IgG, IgM & IgG vaccine titers
  • False + ß-HCG tests d/t presence of heterophile Ab
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24
Q

What is the defect in Common variable immunodeficiency (CVID)?

A

Defect in B-cell maturation

Many causes

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25
Q

How do CVID pts present?

A
  • Can be acquired in 20-30s
  • Inc risk of AI dz
  • Lymphoma
  • Sinopulmonary infections
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26
Q

What are the findings in CVID?

A
  • Normal # of B cells
  • Dec plasma cells
  • Ig
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27
Q

What are the T cell deficiency disorders?

A
  • Thymic aplasia (DiGeorge synd)
  • IL-12 receptor deficiency
  • Hyper-IgE synd (Job’s synd)
  • Chronic mucocutaneous candidiasis
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28
Q

What is the defect in DiGeorge synd?

A

22q11 deletion

Failure to develop 3rd &4th pharyngeal pouches

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29
Q

How do DiGeorge synd pts present?

A
  • Tetany (hypocalcemia)
  • Recurrent viral/fungal infections (T cell def)
  • Congenital heart & great vessel defects
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30
Q

What are the findings in DiGeorge synd?

A
  • Thymus & parathyroids fail to develop→ dec T cells, dec PTH, dec Ca2+
  • Absent thymic shadow on CXR
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31
Q

What is the defect in IL-12 receptor deficiency?

A

Dec Th1 response

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32
Q

How do IL-12 receptor def pts present?

A

Disseminated mycobacterial infections

33
Q

What are the findings in IL-12 receptor def?

A

Dec IFN-gamma

34
Q

What is the defect in Job’s synd?

A

Th1 cells fail to produce IFN-gamma→ inability of neutrophils to respond to chemotactic stimuli

35
Q

How do Job’s synd pts present?

A
  • Coarse facies
  • Cold (noninflammed) staph abscess
  • Retained primary teeth
  • Inc IgE
  • Dermatologic problems (eczema)
36
Q

What are the findings in Job’s synd?

37
Q

What is the defect in Chronic mucocutaneous candidiasis?

A

T-cell dysfunction

38
Q

How do Chronic mucocutaneous candiasis pts present?

A

Candida albicans infections of skin & mucous membranes

39
Q

What are the B & T cell disorders?

A
  • Severe combined immunodeficiency (SCID)
  • Ataxia-telangectasia
  • Hyper-IgM synd
  • Wiskott-Aldrich synd
40
Q

What is the defect in Severe combined immunodeficiency (SCID)?

A

Several types: defective IL-2 receptor (MC, X-linked), adenosine deaminase deficiency

41
Q

How do Severe combined immunodeficiency (SCID) pts present?

A
  • Failure to thrive
  • Chronic diarrhea
  • Thrush
  • Recurrent viral, bacterial, fungal & protozal infections
  • Absence of thymic shadow, germinal centers & B cells
42
Q

What is the tx of SCID?

A

Bone marrow transplant (no allograft rejection)

43
Q

What are the findings in SCID?

A
  • Dec T cell recombinant excision circles (TRECs)
  • Abscence of thymic shadow, germinal centers & T cells
44
Q

What is the defect in Ataxia-telangiectasia?

A

Defects in the ATM gene which codes for DNA repair enzymes

45
Q

What is the triad in Ataxia-telangiectasia?

A
  • Cerebellar defects (ataxia)
  • Spider angiomas (telangiectasia
  • IgA deficiency
46
Q

What are the findings in Ataxia-telangiectasia?

47
Q

What is the defect in Hyper-IgM synd?

A

MC defective CD40L on helper T cells= inability to class switch

48
Q

How do Hyper-IgM synd pts present?

A

Severe pyogenic infections early in life

49
Q

What are the findings in Hyper-IgM synd?

A
  • Inc IgM
  • Dec IgG, IgA, IgE
50
Q

What is the defect in Wiskott-Aldrich synd?

A

X-linked; in WAS gene on X chr→ T cells unable to reorganize actin cytoskeleton

51
Q

What is the triad in Wiskott-Aldrich synd?

A
  • Thrombocytopenic purpura
  • Infections
  • Eczema
52
Q

What are the findings in Wiskott-Aldrich synd?

A
  • Inc IgE, IgA
  • Dec IgM
  • Thrombocytopenia
53
Q

What are the Phagocyte dysfunction disorders?

A
  • Leukocyte adhesion deficiency (type I)
  • Chédiak-Higashi synd
  • Chronic granulomatous dz
54
Q

What is the defect in Leukocyte adhesion def (Type I)?

A

Defect in LFA-1 integrin (CD18) protein on phagocytes

55
Q

How do Leukocyte adhesion def (type I) pts present?

A
  • Recurrent bacterial infections
  • Absent pus formation
  • Delayed separation of umbilical cord
56
Q

What are the findings in Leukocytes adhesion def (type I)?

A

Neutrophilia

57
Q

What is the defect in Chédiak-Higashi synd?

A
  • Autosomal recessive
  • defect in lysosomal trafficking regulator gene (LYST)
  • Microtubule dysfunction in phagosome-lysosome fusion
58
Q

How do Chédiak-Higashi synd pts present?

A
  • Recurrent pygoenic infections by staph & strep
  • Partial albinism
  • Peripheral neuropathy
59
Q

What are the findings in Chédiak-Higashi synd?

A

Giant granules in neutrophils

60
Q

What is the defect in Chronic granulomatous dz?

A

Lack of NADPH oxidase→ dec reactive oxygen species & absent respiratory burst in neutrophils

61
Q

How do Chronic granulomatous dz pts present?

A

Inc susceptibility to catalase + organisms

62
Q

What are the findings in Chronic granulomatous dz?

A
  • ABN dihydrohodamine (DHR) flow cytometry test
  • Nitroblue tetrazolium dye reduction test no longer preferred
63
Q

What is an Autograft?

64
Q

What is a Syngeneic graft?

A

From identical twin or clone

65
Q

What is an Allograft?

A

From nonidentical individual of same species

66
Q

What is a Xenograft?

A

From different species

67
Q

What is the onset after transplantaion in a Hyperacute rejection?

A

Within minutes

68
Q

What is the pathogenesis of Hyperacute transplant rejection?

A

Ab med (type II) b/c of the presence of preformed anti-donor Ab’s in the transplant recipient

69
Q

What are the features of a Hyperacute transplant rejection?

A

Occludes graft vessels causing ischemia & necrosis

70
Q

What is the onset after transplantation in Acute rejection?

A

Weeks later

71
Q

What is the pathogenesis of an Acute transplant rejection?

A

Cell-med d/t CTLs reacting against foreign MHCs. Reversible w/ immunosuppressants

72
Q

What are the features of Acute transplant rejection?

A

Vasculitis of graft vessels w/ dense interstitial lymphocytic infiltrate

73
Q

What is the onset after transplant in Chronic rejection?

A

Months to years

74
Q

What is the pathogenesis of Chronic transplant rejection?

A

Class I-MHCnonself is perceived by CTLs as class I-MHCself presenting a non-self Ag

75
Q

What are the features of Chronic transplant rejection?

A
  • Irreversible
  • T-cell & Ab-med vascular damage
  • Fibrosis of graft tissue & BV’s
76
Q

What is the onset after transplantation in Graft-versus-host rejection?

77
Q

What is the pathogenesis of Graft-versus-host transplant rejection?

A

Grafted immunocompetent T cells proliferate in the irradiated immunocompromised dz host & reject cells w/ “foreign” proteins, resulting in severe organ dysfunction

78
Q

What are the features of Graft-versus-host transplant rejection?

A
  • Maculopapular rash
  • Jaundice
  • Hepatosplenomegaly
  • Diarrhea
  • Usually in BM & liver transplants

Immunology (7 decks)

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