Immunology Flashcards

1
Q

HLA- A, B, C are encoded by MHC Class __. HLA-DR, DP, AD are encoded by MHC Class ___.

A

I, II

remember 1 letter = MHC class I, 2 letters = Class II

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2
Q

MHC Class I mediates _____ immunity while MHC Class II mediates ______ immunity.

A

MHC Class I = CD8+ = viral immunity
(intracellular pathogens)

MHC Class II = CD4+ = bacterial immunity (extracellular pathogens)

Note: there are a few exceptions. For example when a bacteria is intracellular (e.g. listeria), MHC Class I will be used.

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3
Q

MHC II only found on ______ cells.

A

Antigen presenting cells

macrophage, dendritic cell, B cells

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4
Q

What is the mnemonic for remembering which autoimmune diseases are associated with HLA-B27?

A

“PAIR”

Psoriasis
Ankylosing spondylitis
IBD
Reiter’s syndrome

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5
Q

HLA-A3 = ________

A

Hereditary hemochromatosis

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6
Q

HLA-B8 = _______ Disease.

A

Grave’s

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7
Q

HLA-DR2 = What 4 diseases?

A

MS, Hay fever, SLE, Goodpasture’s

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8
Q

DM Type I = HLA-_ and HLA- _

A

DR3 and DR4

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9
Q

RA = HLA-__

A

DR4

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10
Q

What 2 diseases are associated with HLA-DR5?

A

Pernicious anemia

Hashimoto’s

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11
Q

HLA-DR7 = _____ syndrome

A

Steroid-responsive nephrotic syndrome

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12
Q

What are the cytokines which enhance NK cell activity (aka killing of virally-infected and tumor cells)?

A

IL-12
IFN-beta
IFN-alpha

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13
Q

When a dendritic cell picks up a viral pathogen it makes _______ cytokines to stimulate a CD__+ T cell aka _____.

A

Virus -> IFN-alpha, IFN-beta -> CD8+ T cell, aka cytotoxic T lymphocyte

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14
Q

When a dendritic cell picks up an intracellular bacterial pathogen it makes _______ cytokines to stimulate a CD__+ T cell aka _____.

A

Intracellular bacteria -> IFN-gamma, TNF-alpha, IL-2 -> CD4+ TH1 helper T cells

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15
Q

When a dendritic cell picks up a parasitic pathogen it makes _______ cytokines to stimulate a CD__+ T cell aka _____.

A

Parasite -> IL-4, IL-5, IL-13 -> CD4+ TH2 cells which go on to activate B cells to kill the worm

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16
Q

When a dendritic cell picks up a extracellular bacteria and fungus pathogen it makes _______ cytokines to stimulate a CD__+ T cell aka _____.

A

Extracellular bacteria or fungus -> IL-6, IL-17 -> CD4+ TH17 cells which go on to activate B cells to make antibodies against and recruit neutrophils

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17
Q

What does IL-8 do?

A

Neutrophil chemotaxis and phagocytosis upon arrival

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18
Q

The “acute” allergic response is anything that happens within _______.

A

Minutes to hours

Due to pre-formed IgG Abs (Type II hypersensitivity)

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19
Q

What is Chronic Granulomatous Disease? What test is a tip off?

A

X-linked mutation affecting NADPH oxidase which is normally activated within phagocytes to produce ROS AND to activate granule proteases (e.g. elastase, cathepsin G) within phagosomes.

Dysfunction of NADPH oxidase leads to impaired intracellular killing by neutrophils and macrophages leading to recurrent bacterial and fungal infections.

Failure of neutrophils to turn blue upon nitroblue tetrazolium testing indicates CGD.

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20
Q

How does being catalase positive help bacteria evade neutrophil destruction?

A

Catalase destroys hydrogen peroxide that bacteria generate themselves

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21
Q

Recurrent viral, fungal, sinopulmonary, and opportunistic infections (e.g. PJP) is indicative of _____.

A

SCID, or some other immunocompromised state

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22
Q

What is the pathogenesis of sarcoidosis?

A

Sarcoidosis is thought to result from a dysregulated cell-mediated immune response lead by Th1 CD4+ T cells which secrete IL-2 and IFN-gamma. This activates macrophages, promoting non-caseating granuloma formation.

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23
Q

Sarcoidosis is most common in what patient population?

A

African Americans

Especially women 20-39 years old

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24
Q

What are the 2 most common forms of SCID?

A

Most common = X-linked recessive

Second most = adenosine deaminase deficiency (build up of adenosine is cytotoxic to B and T cells).

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25
Q

How do superantigens (e.g. TSS toxin from s. aureus) work to cause hemodynamic collapse?

A

Superantigens cause widespread activation of helper T cells and antigen presenting cells (mainly macrophages) causing widespread T cell activation.

T cells secrete IL-2 cytokines and macrophages secrete IL-1 and TNF causing capillary leakage, hypotension, vascular collapse, skin findings, shock, and multiorgan failure.

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26
Q

What is the most common systemic vasculitis in children? How is it characterized?

A

Henoch-Schonlein purpura (HSP).

Characterized by circulating IgA-antigen immune complexes (Type III hypersensitivity) that deposit in the walls of small vessels and renal mesangium.

Presents as palpable purpura, arthralgias, abdominal pain, possible hematuria.

Tx is generally supportive as it is self-limited unless an intussusception occurs as a result of the GI problems.

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27
Q

Antigens are presented on MHC Class I _____ chains and _____.

A

Heavy chain

Beta 2 microglobulin

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28
Q

How do cytotoxic T cells actually kill?

A

Perforin and granzymes OR

IFN alpha and IFN beta bind to receptors on infected + neighboring cells causing transcription of antiviral enzymes that are capable of halting protein synthesis in the infected cell.

Examples = RNase L and protein kinase E.

One cool thing is that this process only works in the presence of double-stranded RNA (i.e. cells with viral genome present) so the uninfected neighboring cells are able to continue as usual.

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29
Q

Albinism, neurologic defects, nystagmus, immunodeficiency = ?

A

Chediak-Higashi

Autosomal recessive
Characterized by a defect in neutrophil phagosome lysosome fusion causing giant lysosomal inclusions visible on LM and peripheral smear.

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30
Q
Tryptase = \_\_\_\_\_ cells
Myeloperoxidase = \_\_\_\_\_
A
Tryptase = mast cells
Myeloperoxidase = neutrophils
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31
Q

What is the co-stimulatory signal expressed on Th cells for activation?

A

CD40L. They bind to CD40 on B cells to activate them.

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32
Q

Th1 cells regulate a cell-mediated response via

A

IL-2 and IFN-gamma

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33
Q

Th2 cells regulate a humoral-mediated response via

A

IL-4, IL-5

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34
Q

Th1 and Th2 tend to inhibit each other to make a specific immune response via:

A

Th1 makes IL-10

Th2 makes IFN gamma

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35
Q

The ___ chain has VDJ recombination and the ___ chain has VJ combination

A
heavy = VDJ
light = VJ
36
Q

The alternative complement pathway is always ___.

A

on

It is mediated by C3 and held in check by “DAF” on host cells

37
Q

Ig_ and Ig_ are the best at activating complement via the classical complement cascade.

A

IgG and IgM

38
Q

Deficiency of C5-C8 leads to _______ bacteremia.

A

Neisseria

39
Q

What is a helpful mnemonic for remembering cytokines?

A

“Hot T-Bone stEAk”

IL-1: fever (hot)
IL-2: T cells
IL-3: Bone marrow
IL-4: IgE
IL-5: IgA

“clean up on aisle 8” -> neutrophils recruited by IL-8 to clear infections

40
Q

Which cytokines are involved in the adaptive immune system?

A

IL-2
IL-4

IL-5
IL-10

IL-12

2x2=4
5x2=10
12

41
Q

What are the interleukins that induce fever?

A

IL-1
IL-6
TNF-alpha

IL-6 also induces liver production of acute phase reactants

42
Q

Asthma is a Type __ Hypersensitivity

A

I

43
Q

C__ and C__ act as chemoattractants for neutrophils and phagocytes.

A

C3a and C5a

44
Q

Serum sickness is a Type ___ hypersensitivity. IgG and IgM complexes deposit and can cause localized ______ consumption, ultimately leading to hypo______.

A

III

Complement
Hypocomplementemia (decreased serum C3)

45
Q

In general, Type ___ hypersensitivities tend to be limited to an organ while Type ___ involve vessels and are more systemic.

Name examples.

A
II = limited
III = systemic
Type II:
Hemolytic Anemia
Pernicious anemia
Rheumatic fever
Goodpasture's
Grave's
Bullous pemphigoid
Pemphigus vulgaris
Myasthenia Gravis
Type III:
SLE
RA
PAN
PSGN
Serum sickness
Arthus rxn
Hypersensitvity pneumonitis (farmer's lung)
46
Q

_____ syndrome aka thymic aplasia

A

DiGeorge

Absence of thymic shadow
-> decreased T cells

47
Q

Lymphocytes enter the lymph node via the ___ and the spleen via the _____.

A

Lymph node = HEV

Spleen = central arteriole

48
Q

In the spleen the PALS around the central arteriole is the ___ cell zone

A

T cell

49
Q

The marginal zone in the spleen contains _______.

A

Macrophages (antigen presenting cells)

50
Q

Cyclosporine and tacrolimus are _____ inhibitors which _______.

A

Calcineurin inhibitors

Block T cell signaling

51
Q

A bronchoalveolar lavage in sarcoidosis will show an elevated CD_:CD_ ratio which sequesters them so they don’t actually respond to other T cell mediated antigens.

A

CD4 > CD8

52
Q

Three distinguishing features of sarcoidosis from other granulomatous diseases is the presence of:

A

Hypercalcemia (via overactivated 1alpha hydroxylase)
Erythema nodosum on the shins
Anterior uveitis

53
Q

What are patients working in “nuclear and aircraft/aerospace industries” likely to get?

A

Berylliosis from inhaling beryllium dust. Causes a sarcoid-like interstitial lung disease.

54
Q

Which interleukin is responsible for initiating isotype switching?

A

IL-4

Remember TH2 cells activate the humoral immune system via IL-4 and IL-5 secretion

55
Q

Granulomas are formed by what 2 cell types?

A

CD4+ T cells

++++macrophages

56
Q

A blood transfusion in an ABO matched donor can still cause an immediate transfusion reaction if the patient has what deficiency:

A

IgA

and the transfused RBCs will contain IgA

57
Q

Rheumatoid Factor (RF) is an antibody (typically IgM) specific for the ________.

A

Fc component of IgG

RF binds circulating IgG and ACPAs (anti-citrullinated protein antibodies) bind modified self-proteins forming immune complexes that deposit on the synovium and cartilage -> complexes activate complement contributing to chronic inflammation and joint destruction.

58
Q

Positive C-ANCA = ?

A
CREST syndrome (scleroderma)
Wegener's Granulomatosis
59
Q

Positive Anti-dsDNA = ?

What else would be positive in these patients?

A

SLE

+ antiphospholipid antibody (although this can be a stand alone disease)

60
Q

Reactive arthritis = can’t pee, can’t see, can’t climb a tree. What are the more specific symptoms of above mnemonic?

A

Asymmetric oligoarthritis, can be dactylitis or sacroilitis
Conjunctivitis
Urethritis
Hyperkeratotic vesicles of palms and soles

61
Q

What are common infections preceding reactive arthritis?

A

GU: Chlamydia trachomatis
Enteritis: Salmonella, Shigella, Yersinia, Campylobacter, C. diff

62
Q

Although T-cell deficiency would lead to superficial candidiasis (and other fungal infections), disseminated aka hematogenous spread of candida is often due to ________penia.

A

Neutropenia

or otherwise immunocompromised such as receiving chemo

63
Q

What is the cell surface marker for macrophages?

A

CD14

64
Q

Many tumor cells evade the immune system by expressing ______ which downregulates the immune response by inhibiting cytotoxic T cells.

A

PD-1

This is why new monoclonal Abs for tumor suppression bind to PD-1 and block its action.

65
Q

B7 + CD28 = T cell _______

B7 + CTLA4 = T cell ______

A

Activation

Suppression

66
Q

What histopathology is seen in the acute phase of transplant rejection?

A

Dense infiltrate of mononuclear cells (mostly T lymphocytes).

The rejection occurs due to host T-lymphocyte sensitization against graft (foreign) MHC antigens.

67
Q

What immunodeficiency leads to recurrent neisseria infections?

A

Failure of Membrane Attack Complex (MAC) to form (i.e. C5b-C9 of complement cascade).

68
Q

What is ABO disease? When can it occur?

A

Attack of an A or B fetus in an O negative mother. These mothers will have Anti-A and Anti-B antibodies from mimicking antigens in food, bacteria, or other viruses. These are IgGs and can thus cross the placenta and cause hemolysis in the fetus.

Note these antibodies are pre-existing and thus can attack the fetus of a first pregnancy (unlike Rh disease).

When Anti-A and Anti-B are naturally occurring, they are type IgM which cannot cross the placenta.

69
Q

Anti-toposiomerase I (Scl-70) = ?

A

Scleroderma/CREST

70
Q

Positive C-ANCA = ?

A

Scleroderma/CREST

71
Q

Anti-U1 RNP = ?

A

CAN be SLE but most likely mixed connective tissue disorder

72
Q

Anti-Jo-1 = ?

A

Polymyositis or dermatomyositis

73
Q

Polymyositis = _____misial inflammatory infiltrate while dermatomyositis = ______misial.

A
Poly = endo (within muscle fascicle)
Dermato = peri
74
Q

C-ANCA aka ______
Located in the _______
+ in

A

C-ANCA = PR3
Located in the cytoplasm (C)
+ in Wegener’s granulomatosis and CREST

75
Q

P-ANCA aka ______
Located in the _______
+ in _________

A

P-ANCA = MPO
Located in the perinuclear lysosomes
+ in everything else except Wegener’s

76
Q

What is the triad of presenting symptoms of Wegener’s Granulomatosis (aka GPA)?

A

Nose - sinus and septal problems, saddle nose deformity
Lung - Recurrent pneumonitis, interstitial lung disease, alveolar hemorrhage
Kidneys - glomerulonephritis

77
Q

Microscopic polyangiitis MOSTLY affects the ______ but can also affect the ______.

A

Mostly lungs

Some kidney

78
Q

What is the important distinguishing feature of Churg-Strauss?

A

Eosinophilia

often a history of asthma

79
Q

Cryoglobulinemia is a Type ___ hypersensitivity and is cahracterized by:

A

Type III

Antibody immune complexes. One of the most common is IgM clumps, but it can be any Ig.

80
Q

Cryoglobulinemia is associated with Hepatitis ___ and is most often seen in:

A

Hep C

Older women

81
Q

Although Goodpasture’s disease is primarily characterized by linear staining of the basement membrane by Abs, these Abs also upregulate _______ leading to _______.

A

Abs induce complement -> complexes form in the kidneys causing crescentic glomerulonephritis

82
Q

CD3 is found on all mature ___ cells.

A

T

83
Q

X-linked SCID patients are deficient in IL-___ which is needed for preservation of immature B and T cells.

A

IL-7

84
Q

What is the role of the ATM gene in Ataxia-Telangiectasia?

A

Surveillance of DNA damage and stalling the cell cycle to give time for repair -> deficiency = deficient B and T cells

85
Q

What lab findings will you see in Ataxia-Telangiectasia?

A

Especially low serum IgA (why they get so many sinopulmonary infections)
Elevated serum alfa-fetoprotein (AFP)