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Flashcards in Immunology Deck (143):
1

a. What muscles come from first arch?
b. what nerves come from first arch?

a. muscles of mastication, mylohyoid muscle, tensor tympani, tensor veli palatini
b. maxillary and mandibular division of trigeminal nerve

2

What amino acids are found in elastin?

Glycine and proline

3

What is mechanism of adenosine as anti-arrhythmic?

Increases outward K current
Hyper polarization of cells
Decreases intracellular Ca

4

What part of the body drains to:
a. Axillary lymph node
b. Superificial inguinal nodes
c. Popliteal nodes

a. Upper limb, breast, skin above umbilicus
b. anal canal (below pectinate line), skin below umbilicus, scrotum
c. Dorsolateral foot, posterior calf

5

Which part of the GI tract drains to:
a. Celiac lymph nodes
b. Superior mesenteric nodes
c. Inferior mesenteric nodes
d. Internal iliac nodes

a. liver, stomach, spleen, pancreas, upper duodenum
b. lower duodenum, jejunum, ileum, colon to splenic flexure
c. colon from splenic flexure to upper rectum
d. lower rectum to anal canal (above pectinate line), bladder, vagina, prostate

6

Which lymph nodes do the testes drain to? the scrotum?

Testes --> PARA-AORTIC
Scrotum --> superficial inguinal

7

What part of the body does the right lymphatic duct drain?

Right side of body above the diaphragm

8

What part of the body does the thoracic duct drain?

Everything else besides right side of body above diaphragm. Goes into junction of left subclavian and internal jugular veins.

9

Where do you find peer's patches?

In lamina propria submucosa of ileum

10

a. What muscles are derived from second branchial arch?
b. nerves from second branchial arch?

a. Muscles of facial expression, stapedius, stylohyoid
b. Cranial nerve seven

11

Which cells express MHCI?

ALL cells EXCEPT RBCs

12

a. What genes code for MHC I?
b. What genes code for MHC II?

a. HLA-A, B and C
b. HLA-DR, DP, and DQ

13

What diseases does HLA-B27 predispose to?

PAIR
Psoriatic arthritis
Ankylosing spondylitis
Inflammatory Bowel Disease
Reactive arthritis

14

What diseases do HLADR3/4 predispose to?

Diabetes mellitus type I
DR4 - Rheumatoid arthritis

15

a. What is Langerhans Cell Histiocytosis?
b. What markers does it express?
c. ON electron microscopy?
d. How does it present?

a. Group of proliferative disorders of dendritic cells
b. Express S-100 and CD1a
c. Birbeck granules
d. Presents as child with lytic bone lesion and skin rash or recurrent otitis media with mass involving mastoid bone

16

What 4 things are expressed on dendritic cells?

B7
CD40
MHC I
MHC II

17

What mediates the following reactions:
a. hyperacute transplant rejection
b. acute transplant rejection
c. chronic rejection
d. graft vs. host

a. pre-existing recipient antibodies
b. T cells
c. T cells and Antibodies; Cytotoxic T cells treat transplant cells as self cells presenting non-self antigen - irreversible
d. Usually in BM transplants; the grafted immunocompetent T cells proliferate in the immunocompromised host and reject host cells with "foreign proteins"

18

How does graft vs. host disease present?

Maculopapular rash, jaundice
Diarrhea, hepatosplenomegaly

19

Cyclosporine
a. Mechanism
b. Uses
c. Adverse effects (unique one?)

a. Binds to cyclophilin --> inhibits Calcineurin --> prevents production of IL2 --> disrupts T cell activation
b. Prevent transplant rejection, psoriasis, RA
c. Viral infections, lymphomas, NEPHROTOXICITY, HTN

20

Tacrolimus
a. Mechanism
b. Uses
c. Adverse effects

a. Bind FK506 binding protein (FKBP) --> inhibits Calcineurin --> prevents production of IL2 --> blocks T cell activation
b. Transplant rejection prophylaxis
c. Increased risk of diabetes and neurotoxicity,, nephrotoxicity, HTN

21

Sirolimus
a. Mechanism
b. Uses
c. Adverse effects

a. binds FKBP --> inhibits mTOR --> inhibition of T cell proliferation and prevents response to IL2
b. Kidney transplant
c. Not that important - NOT NEPHROTOXIC; anemia, thrombocytopenia, leukopenia

22

Azathioprine
a. Mechanism
b. Use
c. Adverse effects

(precursor to 6-Mercaptopurine)
a. Inhibits lymphocyte proliferation by blocking nucleotide synthesis
b. Transplant rejection prophylaxis (especially kidney), RA, glomerulonephritis
c. BONE MARROW suppression; degraded by Xanthine Oxidase (toxic effects can be increased by Allopurinol)

23

Mycophenolate
a. Mechanism
b. Use

a. Inhibits IMP dehydrogenase --> prevents synthesis of guanine --> no cell replication/proliferation of B cells and T cells
b. Transplant patients and Lupus nephritis

24

Muromonab
a. Mechanism
b. Use

a. Binds CD3 (T cells) --> interferes with T cell signal transduction --> shuts down T cells
b. Transplants

25

Daclizumab
a. Mechanism
b. Use

a. Binds to CD25 (IL2 receptor on activated T cells) --> blocks IL-2 signaling (Sirolimus is similar)

26

Thalidomide
a. mechanism
b. use
c. toxicity

a. affects TNFa
b.Immunosuppression, anti-angiogenic
c. NOT USED - phocomelia, teratogen

27

a. What are the anti-TNFa agents?
b. What are they used for?
c. Which anti-TNF alpha drug is not a monoclonal antibody?

a. Adalimumab, Infliximab
b. Ankylosing spondylitis, RA, IBD, reactive arthritis
c. Etanercept - mimics TNFa receptor (gobbles up TNFa in circulation)

28

Abciximab
a. Mechanism
b. Use

a. Targets platelet glycoproteins IIb/IIIa
b. Anti-platelet agent for prevention of ischemic complications in patients undergoing percutaneous coronary intervention

29

Trastuzumab (Herceptin)
a. Mechanism
b. Use

a. Ab against HER2
b. Breast cancer that expresses HER2

30

Rituximab
a. Mech
b. Use

a. Targets CD20
b. B cell NON-Hodgkin lymphoma

31

Omalizumab
a. Mech
b. Use

a. Targets IgE
b. Severe asthma

32

Bruton agammaglobulinemia
a. defect
b. presentation
c. findings

a. defect in BTK (tyrosine kinase gene) --> no B cell maturation; X linked recessive
b. Recurrent bacterial infections and enteroviral infections after 6 months (decreased maternal IgG)
c. Absent B cells in peripheral blood, decrease IgG of ALL classes; absent lymph nodes/tonsils

33

Selective Ig Deficiency
a. Defect
b. Presentation
c. Findings

a. Most common primary immunodeficiency; unknown
b. Majority asymptomatic, can see airway and GI infections; autoimmune disease (atopy, asthma) anaphylaxis to IgA products
c. Decreased IgA with normal IgG and IgM

34

Common Variable Immunodeficiency
a. Defect
b. Presentation
c. Findings

a. Defect in B cell differentiation
b. Can be acquired in 20-30s, increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections
c. decreased plasma cells, decreased immunoglobulins

35

Thymic aplasia (DiGeorge)
a. defect
b. presentation
c. findings

a. 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches --> absent parathyroids and thymus
b. Cardiac abnormality, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia - recurrent viral, fungal and protozoal infections
c. Decreased T cells, PTH and Calcium; absent thyme shadow on CXR

36

IL-12 receptor deficiency
a. defect
b. presentation
c. findings

a. decreased TH1 response; AR
b. disseminated MYCOBACTERIAL and fungal infections;
c. Decreased IFNy

37

Chronic Mucocutaneous Candidiases
a. defect
b. presentation
c. findings

a. T cell dysfunction; many causes
b. non-invasive Candida albicans infections of skin and mucous membranes
c. absent cutaneous reaction and T cell proliferation in response to Candida antigens

38

Hyper IgM Syndrome
a. defect
b. presentation
c. findings

a. defective CD40L on Th cells --> defect in class switching; X linked recessive (3 types)
b. Severe pyogenic infections early in life; opportunistic infection with Penumocystis, Crypto, CMV
c. Increased IgM, decreased other antibodies

39

SCID
a. defect
b. presentation
c. findings

a. defect in early stem cell differentiation; caused by many including adenosine deaminase deficiency (X linked)
b. failure to thrive, chronic diarrhea, thrush, recurrent viral/bacterial/fungal/protozoal infections (treat with BM transplant)
c. Decreased T cell receptor excision circles (TRECs); absence of thymic shadow, germinal centers, and T cells

40

Wiskott-Aldrich Syndrome
a. defect
b. presentatin
c. findings

a. Mutation in WAS gene (X linked) --> T cells unable to recognize actin cytoskeleton
b. Thrombocytopenia purpura, Eczema (on trunk), Recurrent infections
c. Decreased to normal IgG, IgM; Increased IgE and IgA; fewer/smaller platelets

41

What are the X linked immunodeficiencies?

Wiskott Aldrich
Bruton Agammaglobulinemia
Chronic granulomatous disease
Hyper-IgM syndrome

42

Ataxia Telangiectasia
a.defect
b. presentation
c. findings

a. defects in ATM gene --> failure to repair DNA double strand breaks --> cell cycle arrest
b. Triad = Cerebellar defects (ATAXIA), spider Angiomas (telangiectasia) IgA deficiency
c. Increased AFP, decreased IgA, IgG an IgE; lymphopenia, cerebellar atrophy

43

Chronic Granulomatous Disease
a. defect
b. presentation
c. findings

a. defect in NADPH oxidase --> decreased reactive oxygen species and respiratory burst in neutrophils
b. Increased susceptibility to catalase + organisms (Nocardia, Pseudomonas, Listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia
c. Abnormal dihydroorhodamine test; nitroblue tetrazolium dye reduction test is negative

44

How is CGD treated?

Prophylactic TMP-SMX

45

Chediak-Higashi Disease
a. defect
b. presentation
c. findings

a. defective LYST gene --> defective lysosomal transport --> microtubule dysfunction in pharosome-lysosome fusion (AR)
b. Recurrent pyogenic infections by Staph and Strep; partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis
c. giant granules in granulocytes and platelets, pancytopenia, coagulation defects

46

Leukocyte adhesion deficiency type 1
a. defect
b. presentation
c. findings

a. defect in LFA-1 integrin (CD18) on phagocytes, impaired migration and chemotaxis (AR)
b. recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord
c. Increased neutrophils, absence of neutrophils at infection sites

47

What cytokines do T helper cells produce to stimulate B cells?

IL4, IL5

48

What are the surface markers of B cells?

CD19, CD20, CD21, IgM, IgD

49

What cell markers do T cells have?

CD3, CD28, T cell receptor
Cytotoxic T cells have CD8
Helper T cells have CD4 and CD40L

50

What cell markers do NK cells have?

CD56 and CD16

51

What cell markers do macrophages have?

CD14, MHC II, B7 protein, CD40

52

Which organism's exotoxin works via each of the following mechanisms?
a. inactivates EF2
b. activates Gs
c. Disables Gi
d. Bacterial adenylat cyclase (No G protein involved)
e. Blocks GABA and glycine

a. Corynebacterium diptheriae, Pseudomonas (exotoxin A)
b. Vibrio cholerae, E. coli (ETEC heat labile toxin)
c. Pertussis toxin
d. Bacillus anthracis
e. Clostridium tetani (lockjaw)

53

What drug helps to make diagnosis of Myasthenia gravis?

Edrophonium (acetylcholinesterase inhibitor)
Too short acting to treat

54

What heart sound is associated with dilated CHF?

S3

55

What heart sound is associated with stiffened LV?

S4

56

What is amyloidosis?

Accumulation of one specific protein in the tissues that aggregates into B pleated sheets which causes damage and apoptosis

57

What is AL amyloidosis? What protein is deposited? Which organs does it affect?

(Primary)
Due to deposition of proteins from Ig Light chains
Can occurs as plasma cell disorder or associated with multiple myeloma.
Often affects multiple organ systems including renal (nephrotic syndrome), cardiac (restrictive CM, arrhythmia), hematologic (brushing, splenomegaly), GI (hepatomegaly) and neurologic (neuropathy)

58

What is AA amyloidosis? What protein is deposited? What is it associated with?

(Secondary)
Seen in chronic inflammatory conditions such as RA, IBD, spondyloarthropathy
Fibrils composed of serum Amyloid A; often multi system

59

a. Patient with fibrils made of B2 microglobulin, ESRD and/or long term dialysis.
b. What else may it present as?

a. Dialysis-related amyloidosis
b. Carpal tunnel syndrome

60

Which protein and fibril with each form of amyloidosis?
a. type 2 diabetes
b. Medullary thyroid CA
c. Alzheimer
d. Dialysis -associated

a. amylin, AIAPP
b. Calcitonin, A-CAL
c. B-amyloid, APP
d. B2 microglobulin, AB2M

61

How do you diagnosis amyloidosis on biopsy?

Congo red stain; apple green birefringence

62

Disease associated with anti-IgG antibodies

Rheumatoid arthritis

63

Disease associated with anti-citrullinated protein antibodies (ACPA)

RA

64

Associated with anti-centromere ABs

CREST Scleroderma
(C for centromere, C for CREST)

65

Associated with Anti-Scl-70 Abs

Diffuse Scleroderma
(Scl = Scleroderma)

66

Associated with Anti-histone Abs

Drug-induced Lupus

67

Associated with anti-nuclear Abs (ANA)

SLE

68

Associated with Anti-smith Abs

SLE

69

Associated with Anti-dsDNA Abs

Lupus nephritis

70

Associated with Anti-Jo-1 Abs

Polymyositis/Dermatomyositis

71

Associated with Anti-SSA (anti-Ro)

Sjogren syndrome

72

Associated with Anti-SSB (anti-La)

Sjogren syndrome

73

Associated with Anti-U1-RNP Abs

Mixed CT disease

74

Associated with Anti-desmoglein Abs

Pemphigus vulgaris

75

Associated with Anti-acetylcholine receptor Abs

Myasthenia gravis

76

Associated with Anti-endomysial (anti-tissue transglutaminase)

Celiac disease

77

Associated with Anti-gliadin Abs

Celiac disease

78

Associated with anti-mitochondrial Abs

Primary Biliary Cirrhosis

79

Associated with Anti-smooth muscle Abs

Autoimmune Hepatitis

80

Associated with anti-glutamate decarboxylase Abs

Type 1 Diabetes

81

Associated with Anti-thyrotropin receptor Abs

Graves disease

82

Associated with Anti-thyroid peroxidase (anti-TPO)

Hashimoto thyroiditis

83

Anti-thyroglobulin

Hashimoto thyroiditis, Graves disease

84

Anti-Basement membrane Abs

Goodpasture syndrome

85

c-ANCA

Granulomatosis with polyangiitis (Wegener's)

86

p-ANCA

Pauci-immune crescentic glomerulonephritis
Churg-Strauss syndrome
Microscopic polyangiitis

87

Which vaccines are dangerous for patients with egg allergy?

Influenza
Yellow fever

88

What mediates Type I Hypersensitivity?

Free antigen cross-linkes IgE on presensitized mast cells and basophils --> triggers immediate release of vasoactive amines that act on post capillary venules (histamine and bradykinin)

89

What mediates Type II Hypersensitivity?

Cytotoxic (Ab mediated); IgM an IgG bind to FIXED antigen on "enemy" cell and cause cellular destruction by opsonization/phagocytosis, complement mediated inflammation or Ab-mediated cellular dysfunction

90

What is Serum sickness?

Type III hypersensitivity reaction where antibodies to foreign proteins are produced --> immune complexes form and are deposited in membranes where they fix complement (cause tissue damage); mostly caused by drugs; happens 5-10 days after antigen exposure

91

What are signs of serum sickness?

Fever, urticaria, arthralgia, proteinuria, lymphadenopathy

92

What is Arthus reaction?

Local subacute antibody mediated type III hypersensitivity reaction caused by intradermal injection of antigen; takes 4-12 hours

93

What are signs of Arthus reaction?

Edema, necrosis, activation of complement

94

Acute hemolytic transfusion reaction

Type II

95

Acute rheumatic fever

Type II

96

Arthus reaction

Type III

97

Asthma

Type I

98

Bee sting allergy

Type I

99

Contact dermatitis

Type IV

100

Eczema

Type I

101

Graves disease

Type II

102

Guillain Barre syndrome

Type IV

103

GVHD

Type IV

104

Hashimoto thyroiditis

Type IV

105

ITP

Type II

106

MS

Type IV

107

Myasthenia gravis

Type II

108

PAN

Type III

109

Peanut allergy

Type I

110

Poststreptococcal GN

Type III

111

RA

Type III

112

Seasonal allergic rhinitis

Type I

113

SLE

Type III

114

What is the starting point for the Classic complement pathway?

IgG or IgM recognize C1

115

What is C3b important for?

Opsonization

116

What are the two most important opsonins?

C3b and IgG

117

What are C3a and C5a important for?

Anaphylaxis
C3a - stimulates mast cells and basophils
C5a - neutrophil chemotaxis

118

a. What happens if you are deficient in C1 esterase inhibitor?
b. What drug is C/I?

a. Hereditary angioedema (from elevated Bradykinin)
b. ACE inhibitors are C/I

119

What happens if you are deficient in C3?

Recurrent pyogenic sinus and respiratory infections (Strep pneumo and H influenzae)
Also increased susceptibility to type III hypersensitivity and pyelonephritis

120

What happens if you are deficient in C5b-C9 proteins?

Susceptible to recurrent Neisseria bacteremia

121

a. What is DAF?
b. What happens if you have DAF deficiency?

a. GPI anchored enzyme (CD55)
b. Causes complement mediated lysis of RBCs and Paroxysmal Nocturnal Hemoglobinuria

122

What kinds of organisms are asplenic patients susceptible to?

Encapsulated bacteria

123

Even Some Pretty Nasty Killers Have Shiny Bodies

E. coli
Streptococcus pneumo
Pseudomonas aeruginosa
Neisseria meningitidis
Klebsiella pneumoniae
Haemophilus influenza
Salmonella typhi
Group B Streptococcus

124

Why would patient be missing spleen?

Trauma
Sickle cell patient
Hereditary spherocytosis - RBCs are defective and spleen keeps removing them

125

What are macrophages activated by? What cells produce it?

IFNy
Produced by TH1 and NK cells

126

Which cytokines do macrophages make?

IL1
IL6
TNFa

127

What does IL-12 do?

Induces differentiation of T cells into TH1 cells
Activates NK cells

128

What are the neutrophil chemotactic factors?

IL8, C5a, leukotriene B4

129

What is Aldesleukin? What is it used for?

Recombinant IL-2 used for renal cell carcinoma and metastatic melanoma

130

What are Filgrastim and Sargramostim? What are they used for?

Recombinant GM-CSF used for recovery of bone marrow

131

What is Oprelvekin?

Recombinant IL-11 used for thromobocytopenia

132

What is recombinant IFN-a used for?

Hepatitis B/C, Kaposi sarcoma, malignant melanoma, genital warts, hairy cell leukemia

133

What is recombinant IFN-b used for?

Multiple sclerosis

134

What is recombinant IFN-y used for

Chronic Granulomatous Disease

135

What cytokines do TH1 cells produce?

IL-2 and IFNy

136

What cytokine inhibits macrophage activation?

IL-10

137

What does IL-10 inhibit?

Production of TH1 cells and macrophage activation

138

What inhibits production of TH2 cells?

IFNy

139

What muscles and nerves are derived from third branchial arch?

Stylopharyngeus muscle
Glossopharyngeal nerve (IX)

140

What kind of drug is used in treatment of Myasthenia gravis?

Acetylcholinesterase inhibitors like Pyridostigmine, Physostigmine

141

What cytokines enhance NK cell activity?

IL-12
IL-2
IFN-a
IFN-b

142

What do patients with common variable immunodeficiency have increased risk of later in life?

Autoimmune disease and lymphoma

143

Complications of Sjogren's syndrome

Dental caries (decreased saliva production)
mucosa associated lymphoid tissue lymphoma (UNILATERAL PAROTID ENLARGEMENT)