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Immunodeficiency Flashcards

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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
How do CVID pts present?
* Can be acquired in 20-30s * Inc risk of AI dz * Lymphoma * Sinopulmonary infections
26
What are the findings in CVID?
* Normal # of B cells * Dec plasma cells * Ig
27
What are the T cell deficiency disorders?
* Thymic aplasia (DiGeorge synd) * IL-12 receptor deficiency * Hyper-IgE synd (Job's synd) * Chronic mucocutaneous candidiasis
28
What is the defect in DiGeorge synd?
22q11 deletion Failure to develop 3rd &4th pharyngeal pouches
29
How do DiGeorge synd pts present?
* Tetany (hypocalcemia) * Recurrent viral/fungal infections (T cell def) * Congenital heart & great vessel defects
30
What are the findings in DiGeorge synd?
* Thymus & parathyroids fail to develop→ dec T cells, dec PTH, dec Ca2+ * Absent thymic shadow on CXR
31
What is the defect in IL-12 receptor deficiency?
Dec Th1 response
32
How do IL-12 receptor def pts present?
Disseminated mycobacterial infections
33
What are the findings in IL-12 receptor def?
Dec IFN-gamma
34
What is the defect in Job's synd?
Th1 cells fail to produce IFN-gamma→ inability of neutrophils to respond to chemotactic stimuli
35
How do Job's synd pts present?
* Coarse facies * Cold (noninflammed) staph abscess * Retained primary teeth * Inc IgE * Dermatologic problems (eczema)
36
What are the findings in Job's synd?
Inc IgE
37
What is the defect in Chronic mucocutaneous candidiasis?
T-cell dysfunction
38
How do Chronic mucocutaneous candiasis pts present?
*Candida albicans* infections of skin & mucous membranes
39
What are the B & T cell disorders?
* Severe combined immunodeficiency (SCID) * Ataxia-telangectasia * Hyper-IgM synd * Wiskott-Aldrich synd
40
What is the defect in Severe combined immunodeficiency (SCID)?
Several types: defective IL-2 receptor (MC, X-linked), adenosine deaminase deficiency
41
How do Severe combined immunodeficiency (SCID) pts present?
* Failure to thrive * Chronic diarrhea * Thrush * Recurrent viral, bacterial, fungal & protozal infections * Absence of thymic shadow, germinal centers & B cells
42
What is the tx of SCID?
Bone marrow transplant (no allograft rejection)
43
What are the findings in SCID?
* Dec T cell recombinant excision circles (TRECs) * Abscence of thymic shadow, germinal centers & T cells
44
What is the defect in Ataxia-telangiectasia?
Defects in the *ATM* gene which codes for DNA repair enzymes
45
What is the triad in Ataxia-telangiectasia?
* Cerebellar defects (ataxia) * Spider angiomas (telangiectasia * IgA deficiency
46
What are the findings in Ataxia-telangiectasia?
Inc AFP
47
What is the defect in Hyper-IgM synd?
MC defective CD40L on helper T cells= inability to class switch
48
How do Hyper-IgM synd pts present?
Severe pyogenic infections early in life
49
What are the findings in Hyper-IgM synd?
* Inc IgM * Dec IgG, IgA, IgE
50
What is the defect in Wiskott-Aldrich synd?
X-linked; in WAS gene on X chr→ T cells unable to reorganize actin cytoskeleton
51
What is the triad in Wiskott-Aldrich synd?
* Thrombocytopenic purpura * Infections * Eczema
52
What are the findings in Wiskott-Aldrich synd?
* Inc IgE, IgA * Dec IgM * Thrombocytopenia
53
What are the Phagocyte dysfunction disorders?
* Leukocyte adhesion deficiency (type I) * Chédiak-Higashi synd * Chronic granulomatous dz
54
What is the defect in Leukocyte adhesion def (Type I)?
Defect in LFA-1 integrin (CD18) protein on phagocytes
55
How do Leukocyte adhesion def (type I) pts present?
* Recurrent bacterial infections * Absent pus formation * Delayed separation of umbilical cord
56
What are the findings in Leukocytes adhesion def (type I)?
Neutrophilia
57
What is the defect in Chédiak-Higashi synd?
* Autosomal recessive * defect in lysosomal trafficking regulator gene (LYST) * Microtubule dysfunction in phagosome-lysosome fusion
58
How do Chédiak-Higashi synd pts present?
* Recurrent pygoenic infections by staph & strep * Partial albinism * Peripheral neuropathy
59
What are the findings in Chédiak-Higashi synd?
Giant granules in neutrophils
60
What is the defect in Chronic granulomatous dz?
Lack of NADPH oxidase→ dec reactive oxygen species & absent respiratory burst in neutrophils
61
How do Chronic granulomatous dz pts present?
Inc susceptibility to catalase + organisms
62
What are the findings in Chronic granulomatous dz?
* ABN dihydrohodamine (DHR) flow cytometry test * Nitroblue tetrazolium dye reduction test no longer preferred
63
What is an Autograft?
From self
64
What is a Syngeneic graft?
From identical twin or clone
65
What is an Allograft?
From nonidentical individual of same species
66
What is a Xenograft?
From different species
67
What is the onset after transplantaion in a Hyperacute rejection?
Within minutes
68
What is the pathogenesis of Hyperacute transplant rejection?
Ab med (type II) b/c of the presence of preformed anti-donor Ab's in the transplant recipient
69
What are the features of a Hyperacute transplant rejection?
Occludes graft vessels causing ischemia & necrosis
70
What is the onset after transplantation in Acute rejection?
Weeks later
71
What is the pathogenesis of an Acute transplant rejection?
Cell-med d/t CTLs reacting against foreign MHCs. Reversible w/ immunosuppressants
72
What are the features of Acute transplant rejection?
Vasculitis of graft vessels w/ dense interstitial lymphocytic infiltrate
73
What is the onset after transplant in Chronic rejection?
Months to years
74
What is the pathogenesis of Chronic transplant rejection?
Class I-MHCnonself is perceived by CTLs as class I-MHCself presenting a non-self Ag
75
What are the features of Chronic transplant rejection?
* Irreversible * T-cell & Ab-med vascular damage * Fibrosis of graft tissue & BV's
76
What is the onset after transplantation in Graft-versus-host rejection?
Varies
77
What is the pathogenesis of Graft-versus-host transplant rejection?
Grafted immunocompetent T cells proliferate in the irradiated immunocompromised dz host & reject cells w/ "foreign" proteins, resulting in severe organ dysfunction
78
What are the features of Graft-versus-host transplant rejection?
* Maculopapular rash * Jaundice * Hepatosplenomegaly * Diarrhea * Usually in BM & liver transplants

Immunology (7 decks)

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