Usmle Step 1 Fa Immunology-Immune Responses

Front 1

Il-1
(a) secreted by:
(b) effect:

Back 1

(a) macrophages
(b) induces acute inflammation; chemokine production by mac recruits leukocytes; activates endothelium to express adhesion molecules; endogenous pyrogen

Front 2

IL-2
(a) secreted by:
(b) effect:

Back 2

(a) Th cells
(b) Stimulates growth of helper and cytotoxic T cells

Front 3

Il-3
(a) secreted by:
(b) effect:

Back 3

(a) activated T cells
(b) supports growth/differentiation of bone marrow stem cells (similar to GM-CSF); supports growth and differentiation of myeloid cells

Front 4

IL-4
(a) secreted by:
(b) effect:

Back 4

(a) Th2 cells
(b) promoted differentiation of B cells and enhances class switch to IgG and IgE

Front 5

IL-5
(a) secreted by:
(b) effect:

Back 5

(a) Th2 cells
(b) promotes differentiation of B cells and enhances class switch to IgA. Stimulates production and activation of eosinophils.

Front 6

IL-6
(a) secreted by:
(b) effect:

Back 6

(a) Th cells and macrophages.
(b) Stimulates production of acute phase reactions and Igs.

Front 7

IL-8
(a) secreted by:
(b) effect:

Back 7

(a) macs
(b) Major chemotactic factor for neutrophils

Front 8

IL-7
(a) secreted by:
(b) effect:

Back 8

(a) primary lymphoid organs
(b) stimulates progenitory B and T cell production in the bone marrow

Front 9

IL-10
(a) secreted by:
(b) effect:

Back 9

(a) regulatory T cells (Th2)
(b)suppresses cytokine production of TH1 cells

Front 10

IL-12
(a) secreted by:
(b) effect:

Back 10

(a) B cells and macs
(b) Activates NK and Th1 cells (stimulates cell mediated immunity)

Front 11

IFN gamma
(a) secreted by:
(b) effect:

Back 11

(a) Th1 cells , CTL, NK cells
(b) Stimulates CMI, inhibits TH2, increase expression of class I and II MHC

Front 12

IFN alpha and beta
(a) secreted by:
(b) effect:

Back 12

(a) leukocytes and fibroblasts
(b) inhibits viral protein synthesis by acting on uninfected cells

Front 13

TNF alpha and beta
(a) secreted by:
(b) effect:

Back 13

(a) cell mediated immunity cells
(b) enhances cell mediated immunity

Front 14

Cell surface markers for:
(a) helper T
(b)cytotoxic T
(c) B cells
(d)macs
(e) NK cells
(f) all cells except mature red cells

Back 14

(a) CD4, TCR, CD3, CD28, CD40L
(b) CD8, TCR, CD3
(c) IgM, B7, CD19, CD20, CD21 (receptor for EBV), CD40, MHCII
(d) MHC II, B7, CD40, CD14, receptors for Fc and C3b
(e) receptors for MHCI, CD16 (binds Fc and IgG), CD56
(f) MHC I

Front 15

Effect of C1q, C1r, C1s, C2 or C4 deficiency

Back 15

Marked increase in immune complex diseases, increased infections with pyogenic bacteria.

Front 16

Effect of Factor B, properdin deficiency

Back 16

Increased neisserial infections

Front 17

Effect of C3 deficiency

Back 17

Recurrent bacterial infections (severe, pyogenic sinus and respiratory infections), immune complex disease (type III hypersensitivity)

Front 18

Effect of C5, C6, C7, or C8 deficiency

Back 18

Recurrent meningococcoal and gonococcal infections

Front 19

Effect of C1 esterase inhibitor deficiency

Back 19

Hereditary angioedema

Front 20

Effect of DAF deficiency

Back 20

Leads to complement mediated lysis of RBC's and paroxysmal nocturnal hemoglobinuria

Front 21

Fct of alpha and beta IFNs

Back 21

Inhibit viral protein synthesis

Front 22

Fct of gamma IFNs

Back 22

Increase MHC I and II expression and antigen presentation in all cells

Front 23

Fct of all IFN's

Back 23

Activate NK cells to kill virus infected cells

Front 24

Type I hypersensitivity:
(a) Antibody
(b) Complement?
(c) Effector cells
(d) major examples

Back 24

(a) IgE
(b) basophils, mast cells
(c) no
(d) Hay fever, atopic dermatitis, insert venom sensitivity, anaphylaxis, some food alergies, allergy to animals and animal products, asthma

Front 25

Type II hypersensitivity (cytotoxic)
(a) Antibody
(b) Complement?
(c) Effector cells
(d) major examples

Back 25

(a) IgG, IgM
(b) Yes
(c) PMN, macrophages, NK cells
(d) Autoimmune or drug induced hemolytic anemia, transfusion rxns, HDNB, hyperacute graft rejection, Goodpasture disease, rheumatic fever

Front 26

Type II hypersensitivity (noncytotoxic)
(a) Antibody
(b) Complement?
(c) Effector cells
(d) major examples

Back 26

(a) IgG
(b) No
(c) Non
(d) Myasthenia gravis, Grave's Disease, TIIDM

Front 27

Type III hypersensitivity
(a) Antibody
(b) Complement?
(c) Effector cells
(d) major examples

Back 27

(a) IgG, IgM
(b) Yes
(c) PMN, macrophages
(d) SLE, RA, PAN, PSGN, Arthus rxn, serum sickness

Front 28

Type IV hypersensitivity
(a) Antibody
(b) Complement?
(c) Effector cells
(d) major examples

Back 28

(a) none
(b) no
(c) CTL, Th1, macs
(d) TB test, TB, leprosy, Hashimoto thyroiditis, poison ivy (contact dermatitis), acute graft rejection, GVHD, IDDM

Front 29

Chronic granulomatous disease
(a) defect
(b) symptoms
(c) diagnosis

Back 29

(a) deficiency of NADPH oxidase (any of the 4 component proteins)l failure to generate superoxide anion and other oxygen radicals
(b) recurrent infections with catalase positive bacteria and fungi; marked susceptibility to opportunistic infections (S. aureus, E.coli, and Aspergillus).
(c) negative nitroblue tetrazolium dye reduction test

Front 30

Chediak-Higashi Syndrome
(a) defect
(b) symptoms
(c) inheritence

Back 30

(a) granule structural defect; microtubule and subsequent lysosomal emptying dysfct
(b) recurrent bacterial infx; peripheral neuropathy; absent NK activity, partial albinism
(c) AR

Front 31

Leukocyte adhesion deficiency
(a) defect
(b) symptoms

Back 31

(a) Absence of CD18 (common beta chain on the leukocyte integrins)
(b)presents early with recurrent bacterial infx, absent pus formation, neutrophilia, and delayed sep of umbilicus

Front 32

Macrophage chemotactic factors

Back 32

C5a, MCP-1, MIP-1alpha, PDGF, TGF alpha

Front 33

Bruton hypoagammaglobulinemia
(a) defect
(b) signs/symptoms
(c) inheritence

Back 33

(a) Deficiency of tyrosine kinase blocks B cell maturation
(b) Low Ig of all classes, no circulating B cells, but pre B cells in bone marrow in normal numbers, and normal cell mediated immunity
(c) XLR

Front 34

Selective IgA deficiency
(a) defect
(b) signs/symptoms

Back 34

(a) Deficiency of IgA (most common)
(b) Repeated sinopulmonary and GI infections; milk allergiesa dn diarrhe are common. Anaphylaxis upon exposure to blood products w/IgA

Front 35

X linked hyper IgM syndrome
(a) defect
(b) Symptoms/Signs

Back 35

(a) CD40L on activated T cells
(b) High serum IgM without other isotypes. Normal B and T #, susceptibility to bacteria and opportunists ; presents early with severe pyogenic infx.

Front 36

Common variable immunodeficiency (CVID)
(a) defect
(b) Symptoms/Signs

Back 36

(a) B cell maturation defect and hypogammaglobulinemia
(b) Both sexes affected, childhood onset, recurrent bacterial infx and incr susceptbility to giardia. Incr risk later in life to autoimmune disease, lymphoma or gastric cancer. Normal # of circulating B cells but low # of plasma cells.

Front 37

DiGeorge's Syndrome
(a) defect
(b) clinical manifestations

Back 37

(a) failure of formation of 3rd and 4th pharyngeal pouches resulting in thymic aplasia
(b) Facial abnormalities, hypoparathyroidism, cardiac malformations, depression of T cell #, absence of T cell response; recurrent viral and fungal infx due to lack of T cell deficiency.

Front 38

MHC Class I deficiency
(a) defect
(b) clinical manifestations

Back 38

(a) Failure of TAP1 molecules to transport peptides to ER
(b) CD8+ cells deficient, CD4+ T cells normal, recurring viral infx, normal DTH, normal Ab production

Front 39

Wiskott-Aldrich syndrome
(a) defect
(b) clinical manifestations

Back 39

(a) Defect in cytoskeletal glycoprotein, x linked; inability to mount IgM response to capsular polysacs of bacteria. Associated with high IgE and IgA.
(b) Defective responses to bacterial polysacc and depressed IgM, gradual loss of humoral and cellular response, thrombocytopenia and exzema

Front 40

Ataxia telangiectasia
(a) defect
(b) clinical manifestations

Back 40

(a) Defect in kinase involved in the cell cycle; Defect in DNA repair enzyme w/ assoc IgA deficiency.
(b) Ataxia (gait abnormalities), telangiectasia (capillary distortions in eye), deficiency of IgA, and IgE production.

Front 41

Severe combined immunodeficiency (SCID)
(a) Defect(s)
(b) clinical manifestations

Back 41

(a)
1-defects in common gamma chain of IL2 receptor
2-Adenosine deaminase deficiency (results in toxic metabolic products in cells)
3-defect in signal transduction from T cell, IL-2 receptors
4-Bare lymphocyte syndrome/MHC II deficiency
(b) 1-3: chronic diarrheal skin, mouth, throat, lesions; opportunistic (fungal infx); low levels of circulating lymphocytes; cells unresponsive to mitogens.
4-T cells present and responsive to nonspecific mitogens, no GVHD, deficient CD4+ T cells, hypogammaglobulinemia.

Front 42

Job's Syndrome
(a) Defect
(b) clinical manifestatioins

Back 42

(a) Failure of IFN gamma production by helper T cells and subsequent neutrophils failure to respond to chemotactic stimuli
(b) coarse faces, cold staph abscesses, retained primary teeth, increased IgE and dermatologic problems (exzema).

Front 43

Associated disorder: antinuclear antibodies

Back 43

SLE

Front 44

Associated disorder:anti dsDNA, anti smith

Back 44

Specific for SLE

Front 45

Associated disorder: antihistone

Back 45

Drug induced lupus

Front 46

Associated disorder: anti IgG

Back 46

Rheumatoid factor (RA)

Front 47

Associated disorder: Anticentromere

Back 47

Scleroderma (CREST)

Front 48

Associated disorder: anti-Scl-70

Back 48

Scleroderma (diffuse)

Front 49

Associated disorder: Antimitochondrial

Back 49

Primary biliary cirrhosis

Front 50

Associated disorder: antigliadin, antiendomysial

Back 50

Celiac disease

Front 51

Associated disorder: anti basement membrane

Back 51

Goodpasture's disease

Front 52

Associated disorder: anti desmoglein

Back 52

Pemphigus vulgaris

Front 53

Associated disorder: antimicrosomal, antithyroglobulin

Back 53

Hashimoto's thyroiditis

Front 54

Associated disorder: Anti Jo 1

Back 54

Polymyositis, dermatomyositis

Front 55

Associated disorder: anti SS A (anti Ro)

Back 55

Sjogren's syndrome

Front 56

Associated disorder: anti SS B (anti La)

Back 56

Sjogren's syndrome

Front 57

Associated disorder: anti U1 RNP (anti ribonucleoprotein)

Back 57

Mixed connective tissue disease

Front 58

Associated disorder: anti smooth muscle

Back 58

Autoimmune hepatitis

Front 59

Associated disorder: anti glutamate decarboxylase

Back 59

Type I DM

Front 60

Associated disorder: c-ANCA

Back 60

Wegner's granulomatosis

Front 61

Associated disorder: p-ANCA

Back 61

Other vasculitides

Front 62

Associated disorder(s): HLA A3

Back 62

Hemochromatosis

Front 63

Associated disorder(s): B27

Back 63

Ankylosing spondylitis, Reiter's, Inflammatory Bowel Disase, Psoriasis

Front 64

Associated disorder(s): B8

Back 64

Grave's disease

Front 65

Associated disorder(s): DR2

Back 65

Multiple sclerosis, hay fever, SLE, Goodpasture's

Front 66

Associated disorder(s): DR3

Back 66

Diabetes mellitus type I

Front 67

Associated disorder(s): DR4

Back 67

Rheumatoid arthritis, DM type I

Front 68

Associated disorder(s): DR5

Back 68

Pernicious anemia, Hashimoto's

Front 69

Associated disorder(s): DR7

Back 69

Steroid responsive nephrotic syndrome

Front 70

Hyperacute rejection
(a) cell type involved
(b) time profile
(c) describe mechanism

Back 70

(a) none (preformed Ab)
(b) minutes after transplant
(c) preformed antidonor Abs in transplant recipient

Front 71

Acute rejection
(a) cell type involved
(b) time profile
(c) describe mechanism

Back 71

(a) cytotoxic T lymphocytes
(b) weeks after transplant
(c) T cells reacting against foreign MHC's (reversible w/cyclosporine and OKT3)

Front 72

Chronic rejection
(a) cell type involved
(b) time profile
(c) describe mechanism

Back 72

(a) T and B cell
(b) months to years after
(c) Vascular damage (obliterative fibrosis). Irreversible. Class I MHC non self is perceived by CTL's as class I MHC self presenting a non self antigen.

Front 73

GVHD
(a) cell type involved
(b) describe mechanism
(c) symptoms

Back 73

(a) Immunocompetent T cells
(b) T cells proliferate in irradiated IC host and reject cells w/"foreign" proteins, resulting in severe organ dysfct.
(c) Maculopapular rash, jaundice, HSM, and diarrhea

Front 74

Cyclosporine
(a) mechanism
(b) clinical use
(c) toxicity

Back 74

(a) Binds to cyclophilins. Complex blocks differentiation and activation of T cells by inhibiting calcineurin preventing production of IL-2 and it's receptor.
(b) Suppresses organ rejection after transplant; select autoimmune disorders
(c) predispose to viral infx and lymphoma; nephrotoxic (but preventable w/mannitol diuresis)

Front 75

Tacrolimus (FK506)
(a) mechanism
(b) clinical use
(c) toxicity

Back 75

(a) similar to cyclosporine; binds to FK binding protein, inhibiting secretion of IL2 and other cytokines
(b) potent immunosuppressive used in organ transplant patients
(c) significant-nephrotoxicity, peripheral neuropathy, HTN, pleural effusion, hyperglycemia

Front 76

Azathioprine
(a) mechanism
(b) clinical use
(c) toxicity

Back 76

(a) antimetabolite precusor of 6 mercaptopurine that interferes w/metabolism and synthesis of nucleic acids; toxic to proliferating lymphocytes
(b) kidney transplantation, autoimmune disorders (including glomerulonephritis and hemolytic anemia)
(c) BM suppression. Active metabolite mercaptopurine is metabolized by xanthine oxidase, thus toxic effects may be increased by allopurinol

Front 77

Muromonab-CD3
(a) mechanism
(b) clinical use
(c) toxicity

Back 77

(a) Monoclonal ab that binds Cd3 (episolon chain) on surface of T cells. Blocks cellular interaction w/CD3 protein responsible for T cell signal transduction
(b) Immunosuppression after kidney transplant
(c) cytokine release syndrome; hypersensitivity rxn

Front 78

Sirolimus (rapamycin)
(a) mechanism
(b) clinical use
(c) toxicity

Back 78

(a) Binds to mTOR which inhibits T cell proliferation in response to IL2
(b) immunosuppression after kidney transplantation in combo with cyclosporine and corticosteroids
(c) hyperlipidemia, thrombocytopenia, leukopenia

Front 79

Mycophenolate mofetil: mechanism

Back 79

Inhibits de novo guanine synthesis and blocks lymphocyte production

Front 80

Daclizumab mechanism

Back 80

Monoclonal Ab w/high affinity for IL2 receptor on activated T cells

Front 81

Clinical use of recombinant cytokine: aldesleukin (IL-2)

Back 81

Renal cell carcinoma, metastatic melanoma

Front 82

Clinical use of recombinant cytokine: erythropoietin

Back 82

Anemias (esp renal failure)

Front 83

Clinical use of recombinant cytokine:Filgrastim (GCSF)

Back 83

Recovery of bone marrow

Front 84

Clinical use of recombinant cytokine:Sargramostim (GM CSF)

Back 84

Recovery of bone marrow

Front 85

Clinical use of recombinant cytokine: Alpha IFN

Back 85

hepB,C, Kaposi's sarcoma, leukemias, malignant melanomas

Front 86

Clinical use of recombinant cytokine: Beta IFN

Back 86

Multiple sclerosis

Front 87

Clinical use of recombinant cytokine: Gamma IFN

Back 87

Chronic granulomatous disease

Front 88

Clinical use of recombinant cytokine: Oprelvekin (IL-11)

Back 88

Thrombocytopenia

Front 89

Clinical use of recombinant cytokine: Thrombopoietin

Back 89

Thrombocytopenia