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Diseases and Syndromes > First-Aid: Immunology > Flashcards

First-Aid: Immunology Flashcards

1
Q

Palivizumab

A

Target: RSV F protein
Use: RSV prophylaxis for infants
vizu virus

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2
Q

Omalizumab

A

Target: IgE
Use: Allergic asthma, prevents IgE binding to FceRI

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3
Q

Digoxin immune Fab

A

Target: Digoxin
Use: Antidote for digoxin toxicity

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4
Q

Denosumab

A

Target: RANKL
Use: Osteoporosis, inhibits osteoclast maturation (mimics osteoprotegrin)
OS osteoclasts

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5
Q

Abciximab

A

Target: Glycoprotein IIb/IIIa
Use: Anti-platelet, prevents ischemia in coronary intervention
IIb x IIIa = “cix”

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6
Q

Infliximab and adalimumab

A

Target: TNF-a
Use: IBD, rhematoid arthritis, ankylosing spondylitis, psoriasis
RA “inflix” pain in “da lim”bs

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7
Q

Natalizumab

A

Target: a4-integrin
Use: MS, Chron disease
Risk: PML in patients with JC virus
(a4-integrin facilitates leukocyte adhesion)

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8
Q

Trastuzumab

A

Target: HER2/neu
Use: Breast cancer, gastric cancer
HER2 – “2”

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9
Q

Rituximab

A

Target: CD20
Use: B-cell NH’s lymphoma, rheumatoid arthritis (with MTX), ITP

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10
Q

Cetuximab

A

Target: EGFR
Use: Stage IV colorectal cancer, head and neck cancer

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11
Q

Bevacizumab

A

Target: VEGF
Use: Colorectal cancer, renal cell carcinoma

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12
Q

Alemtuzumab

A

Target: CD52
Use: CLL
“lem” for lymph

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13
Q

IFN-y

A

Use: chronic granulomatous disease

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14
Q

IFN-B

A

Use: Multiple sclerosis

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15
Q

IFN-a (x8)

A

Use: Chronic Hep B and Hep C, Kaposi sarcoma, hairy cell leukemia, condyloma acuminatum, renal cell carcinoma, malignant melanoma

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16
Q

Aldesleukin (interleukin-2)

A

Use: Renal cell carcinoma, metastatic melanoma

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17
Q

Sargramostim (granulocyte-macrophage colony stimulating factor)

A

Use: recovery of bone marrow

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18
Q

Filgrastim (granulocyte colony-stimulating factor)

A

Use: recovery of bone marrow

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19
Q

Oprelvekin (interleukin-1)

A

Use: Thrombocytopenia

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20
Q

Thrombopoietin

A

Use: Thrombocytopenia

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21
Q

Epoetin alfa (erythropoietin)

A

Use: anemias (especially in renal failure)

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22
Q

Glucocorticoids

A

Target: NF-kB, decrease transcription, suppress B/T cells
Use: Transplant rejection prophylaxis , many autoimmune disorders, inflammation
Tox: Hyperglycemia, osteoporosis, central obesity, muscle breakdown, psychosis, acne, hypertension, cataracts, peptic ulcers
Note: Can cause iatrogenic Cushing syndrome

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23
Q

Azathioprine

A

Mech: antimetabolite precursor of 6-mercaptopurine, blocks nucleotide synthesis and inhibits lymphocyte proliferation
Use: Transplant rejection prophylaxis, RA, Chron, glomerulonephritis, other autoimmune
Tox: Leukopenia, anemia, thrombocytopenia
Note: toxicity increased by allopurinol, since 6-MP is degraded by xanthine oxidase
“purine” “prine”

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24
Q

Basiliximab

A

Mech: monoclonal Ab to IL-2R
Use: Kidney transplant rejection prophylaxis
Tox: Edema, HTN, tremor

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25
Q

Sirolimus (Rapamycin)

A

Mech: mTOR inhibitor, binds FKBP. Prevents IL-2 signal transduction, thereby blocking T-cell activation and B cell differentiation.
Use: Kidney transplant rejection prophylaxis
Tox: Anemia, thrombocytopenia, leukopenia, insulin resistance, hyperlipidemia. NOT nephrotoxic.
Note: Kidney “sir-vives” Synergistic with cyclosporine. Used in drug eluting stents.

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26
Q

Tacrolimus

A

Mech: Calcineurin inhibitor, binds FK506 binding protein (FKBP), blocks T-cell activation by preventing IL-2 transcription.
Use: transplant rejection prophylaxis
Tox: Diabetes and nephrotoxicity, similar to cyclosporine but without gingival hyperplasia or hirsutism
Note: “-limus” drugs bind FKBP

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27
Q

Cyclosporine

A

Mech: Calcineurin inhibitor, binds cyclophilin, blcoks T-cell activation by preventing IL-2 transcription.
Use: transplant rejection prophylaxis, psoriasis, RA
Tox: Nephrotoxicity, HTN, hyperlipidemia, hyperglycemia, tremor, hirsutism, gingival hyperplasia

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28
Q

Hyperacute rejection

A

Onset: minutes
Mech: Recipient antibodies, Type II reaction, activation of complement
Path: Widespread thrombosis –> ischemia and necrosis
Treat: graft must be removed

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29
Q

Acute rejection

A

Onset: Weeks to months
Mech: Cellular or humoral. Cytotoxic T-cell react to donor MHCs, or host antibodies develop after transplant, leading to a syndrome similar to that seen in hyperacute
Path: Vasculitis of graft vessels with dense interstitial lymphocytic infiltrate
Treat: prevent/reverse with immunosuppression

30
Q

Chronic rejection

A

Onset: months to years
Mech: Recipient MHC percieve donor MHC as recipient and react against donor antigens presented. has both cellular and humoral components.
Path: Heart - atherosclerosis, Lungs - bronchiolitis, Liver - vanishing bile ducts, Kidney - vascular fibrosis and glomerulopathy
Irreversible damage.

31
Q

Graft-vs-host disease

A

Onset: variable
Mech: Graft T-cells proliferate in immunocompromised host and attack host cells
Path: Sever organ dysfunction. Maculopapular rash, jaundice, diarrhea, HSM.
Notes: Often found in bone marrow and liver transplants (rich in lymphocytes). Can be beneficial in leukemia (graft-vs-tumor effect)

32
Q

Chronic granulomatous disease

A

Immune def: phagocyte decreased ROS. NADPH oxidase defect, absent respiratory burst in neutrophils, X-linked recessive.
Presents: Increased sus. to catalase + organisms (PLACESS)
Findings: Abnormal flow cytometry dihydrohodamine test. Negative tetrazolium dye reduction test (out of favor).

33
Q

Catalase + organisms (PLACESS)

A

Pseudomonas, listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia

34
Q

Chediak-Higashi syndrome

A

Immune def: phagocyte microtubule dysfunction in phagosome-lysosome fusion. Defective LYST gene, autosomal recessive.
Presents: Recurrent pyogenic infections by staph and strep. Partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis.
Findings: giant granules in neutrophils and platelets. Pancytopenia, mild coag defects.

35
Q

Leukocyte adhesion deficiency (Type 1)

A

Immune def: phagocyte impaired migration and chemotaxis, impaired LFA-1 integrin (CD18) on lymphocytes, autosomal recessive.
Presents: Recurrent bacterial skin/mucosal infections. Absent pus formation, impaired healing. Delayed separation of umbilical cord (>30 days).
Find: Increased neutrophils, but absence of neurtophils at infection sites

36
Q

Wiskott-Aldrich syndrome

A

Immune def: T-cells unable to recognize actin cytoskeleton. WAS gene, X-linked recessive.
Presents: (WATER) Thrombocytopenia, eczema, recurrent infections. Increased risk of autoimmunity and malignancy.
Find: Elevate IgE and IgA, low-normal IgM and IgG. Fewer and smaller platelets.

37
Q

Hyper-IgM syndrome

A

Immune def: Th cells, class switching defect, defective CD40L. x-linked recessive.
Presents: Sever pyogenic infections in early life. Opportunistic Pneumocystis, Cryptosporidium, and CMV.
Find: Elevated IgM, Massively decreased IgG, IgA and IgE

38
Q

Ataxia-telangiectasia

A

Immune def: T-cell cell cycle arrest. Defect in ATM gene, so DNa double strand breaks cause arrest.
Presents: (AAA-triad) cerebellar defects, spider angiomas, and IgA deficiency.
Find: Increased AFP, decreased IgA, IgG, IgA. Lymphopenia, cerebellar atrophy.

39
Q

Severe Combined Immunodeficiency (SCID)

A

Immune def: T-cell disorder, several types.
1) IL-2R gamma chain defect, X-linked
2) adenosine deaminase deficiency (autosomal recessive)
Presents: Failure to thrive, chronic diarrhea, thrush, recurrent infections of all sorts. Treat with bone marrow transplant. There won’t be any rejection, so that’s cool.
find: Decreased T-cell receptor excision circles. Absent thymic shadow on CXR, absent germinal centers on lymph node biopsy, and absent T-cells on flow.

40
Q

Chronic mucocutaneous candidiasis

A

Immune def: T-cell dysfunction, many causes
Presents: Candida infections on skin and mucous
Find: No T-cell proliferation or cutaneous reaction in response to Candida.

41
Q

Autosomal dominant hyper IgE syndrome (Job syndrome)

A

Immune def: Th17 cell deficiency due to STAT3 mutation -> impaired recruitment of neutrophils
Presents: (FATED) coarse facies, cold staph abscesses, retained primary teeth, increase in IgE, Dermatologic problems (eczema).
Find: Increased IgE, decreased IFN-y

42
Q

IL-12 receptor deficiency

A

Immune def: decreased Th1 response, autosomal recessive.
Presents: Disseminated fungal and mycobacterial infections, sometimes after admin of BCG vaccine.
Find: Decreased IFN-y

43
Q

Thymic aplasia (DiGeorge syndrome)

A

Immune def: failure of 3rd and 4th pharyngeal pouches with absent thymus and parathyroids, 22q11 deletion
Presents: Tetany (hypocalcemia), recurrent viral/fungal infectons (T-cell mediated), other conotruncal abnormalitites (ToF, truncus arteriosis)
Find: decreased T-cells, decreased PTH, decreased calcium. Absent thymic shadow on CXR. Detect 22q11 mutation on FISH.

44
Q

Common variable immunodeficiency

A

Immune def: B-cell differentiation defect. Many causes.
Presents: can be acquired mid-life, increased risk of autoimmune, bronchiectasis, lymphoma, sinopulmonary infections
Find: decreased plasma cells, decreased immunoglobulins

45
Q

Selective IgA deficiency

A

Immune def: Unknown mechansim. Decreased IgA (

46
Q

X-linked (Bruton) agammaglobulinemia

A

Immune def: No B-cell maturation. Defect in BTK gene (a tyrosine kinase), X-linked recessive
Presents: Recurrent bacterial and enteroviral infections after 6 months post-birth.
Find: Normal CD19 B-cell count, but decreased pro-B, Ig of all classes, and scanty lymph nodes and tonsils.

47
Q

Lack of B-cells makes patient vulnerable to…?

A

Encapsulated organisms (SHiNE SKiS)
Strep pneum, Haemophilus influenza B, Neisseria mening, E coli, Salmonella, Klebsiella pneum, group B Strep
AND
Enteroviral encephalitis, poliovirus (DO NOT GIVE LIVE VACCINE)
AND
GI giardiasus, since no IgA. Bacterial infections predominate

48
Q

Lack of T-cells makes patient vulnerable to…?

A

Sepsis AND
CMV, JCV, EBV, VZV, and chronic respiratory/GI viruses AND
Candida, PCP

49
Q

Lack of granulocytes makes patient vulnerable to…?

A

Staph, Burkholderia cepacia, Serratia, Nocardia, AND Candida, Aspergillus

50
Q

Lack of complement makes patient vulnerable to…?

A

Neisseria (since no membrane attack complex)

51
Q

Anti-ACh receptor Abs

A

Myasthenia gravis

52
Q

Anti-basement membrane Abs

A

Goodpasture syndrome

53
Q

Anti-cardiolipin Abs, lupus anticoagulant

A

SLE, antiphospholipid syndrome

54
Q

Anticentromere Abs

A

Limited scleroderma (CREST syndrome)

55
Q

Anti-desmoglein Abs

A

Pemphigus vulgaris

56
Q

Anti-dsDNA ab, anti-smith Abs

A

SLE (these are both subtypes of ANAs)

57
Q

Anti-glutamate decarboxylase Abs

A

Type 1 DM

58
Q

Anti-hemidesmosome Abs

A

Bullous pemphigoid

59
Q

Antihistone Abs

A

Drug-induced lupus

60
Q

Anti-Jo-1, anti-SRP, anti-Mi-2 Abs

A

Polymyositis, dermatomyositis

61
Q

Antimicrosomal, antithyroglobulin Abs

A

Hashimoto thyroiditis

62
Q

Antimitochondrial Abs

A

1* biliary cirrhosis

63
Q

Antinuclear Abs

A

SLE, nonspecific

64
Q

Anti-Scl-70 (anti-DNA topoisomerasa 1) Abs

A

Scleroderma (diffuse)

65
Q

Anti-smooth muscle

A

Autoimmune hepatitis

66
Q

Anti-SSA, anti-SSB (anti-Ro, anti-La) Abs

A

Sjogren syndrome (no tears or saliva or lubrication)

67
Q

Anti-TSH receptor Abs

A

Graves disease

68
Q

Anti-U1 RNP (ribonucleoprotein) Abs

A

Mixed connective tissue disease

69
Q

c-ANCA (PR3-ANCA)

A

Granulomatosis with polyangiitis (Wegener)

70
Q

IgA antiendomysial, IgA anti-tissue translgutaminase

A

Celiac disease

71
Q

p-ANCA (MPO-ANCA)

A

Microscopic polyangiitis, Churg-Strauss syndrome

72
Q

Rhematoid factor (antibody, most often IgM, specific to IgG Fc region, anti-CCP

A

Rhuematoid arthritis

Diseases and Syndromes (5 decks)

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