Hypersensitivity & Immunodeficiency Flashcards

1
Q

Type 1 hypersensitivity is [time frame], [Ig]-mediated, and can lead to anaphylaxis

A

Type 1 hypersensitivity is immediate, IgE-mediated, and can lead to anaphylaxis

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2
Q

Type 1 hypersensitivities always begin with [stage]

A

Type 1 hypersensitivities always begin with sensitization

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3
Q

Asthma and allergic rhinitis are examples of [Type] hypersensitivity

A

Asthma and allergic rhinitis are examples of Type 1 hypersensitivity

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4
Q

In a type 1 hypersensitivity reaction, mast cells are activated by _

A

In a type 1 hypersensitivity reaction, mast cells are activated by cross-linking of bound IgE

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5
Q

The immediate response in a T1 hypersensitivity reaction involves activation of mast cells followed by release of _

A

The immediate response in a T1 hypersensitivity reaction involves activation of mast cells followed by release of pre-formed granules (histamine, proteases)
* This immediate phase is what can cause anaphylactic shock

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6
Q

Histamine effects include:
* Smooth muscle _
* Vascular permeability _
* Vascular smooth muscle tone _
* Mucus secretion _
* Pruritus

A

Histamine effects include:
* Smooth muscle contraction (wheezing)
* Vascular permeability increases (swelling, hives)
* Vascular smooth muscle tone decreases (hypotension)
* Mucus secretion increases
* Pruritus

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7
Q

Also part of the immediate response, _ and _ are synthesized and act as potent bronchial constrictors and increase vascular permeability

A

Also part of the immediate response, prostaglandins and leukotrienes are synthesized and act as potent bronchial constrictors and increase vascular permeability
* Neutrophils and macrophages also get recruited to the site

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8
Q

The release of [cytokine] from Th2 cells stimulates eosinophils

A

The release of IL-5 from Th2 cells stimulates eosinophils

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9
Q

The late phase of a T1 hypersensitivity reaction is focused on synthesizing _ to further amplify the immune response

A

The late phase of a T1 hypersensitivity reaction is focused on synthesizing cytokines to further amplify the immune response

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10
Q

Mast cells make [cytokines] in the late phase

A

Mast cells make IL-4, IL-5, IL-13, TGF-b in the late phase
* IL-5 recruits eosinophils which further tissue damage

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11
Q

(True/False) Chronic urticaria is Ig-E mediated

A

False; if the patient has hives daily/ continuously it cannot be IgE-mediated
* IgE mediated means immediate response –> degranulation of mast cells
* It then takes time to make granules again

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12
Q

In order to diagnose anaphylaxis, there must be signs of _

A

In order to diagnose anaphylaxis, there must be signs of 2 systems involved
1. Cutaneous
2. Respiratory
3. GI
4. Cardiovascular

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13
Q

(True/False) Only use Epi when a patient has respiratory involvement

A

False; it is anaphylaxis once two systems are involved- don’t wait to use Epi

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14
Q

Epinephrine treats anaphylaxis via its affect as an _

A

Epinephrine treats anaphylaxis via its affect as an alpha and beta agonist
* In addition to treating hypotension and bronchoconstriction it mitigates some mast cell degranulation directly via unknown mechanism

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15
Q

Why are anti-histamines and steroids not indicated for treatment of anaphylaxis?

A

Why are anti-histamines and steroids not indicated for treatment of anaphylaxis?
* Anti-histamines only help the pruritus symptoms and steroids do not work acutely

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16
Q

Steroids are more helpful in the late phase of T1 hypersensitivity; explain

A

Steroids affect transcription in the nucleus which takes time, only helps in the late phase to reduce cytokine synthesis

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17
Q

_ is a monoclonal antibody that binds free IgE to prevent its binding to mast cells and basophils; it can be given to patients with allergies to improve reaction threshold

A

Omalizumab is a monoclonal antibody that binds free IgE to prevent its binding to mast cells and basophils; it can be given to patients with allergies to improve reaction threshold

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18
Q

The first line treatment for allergic rhinitis is _

A

The first line treatment for allergic rhinitis is intranasal steroid (fluticasone, budesonide, triamcinolone)
* Most effective if taken over time, does not work immediately

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19
Q

Azelastine and olopatadine are [drug category] which are second-line options for allergic rhinitis

A

Azelastine and olopatadine are intranasal antihistamines which are second-line options for allergic rhinitis
* Work immediately but only 50% as effective as intranasal steroids

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20
Q

Cetirizine, fexofenadine, and loratadine are [drug category] which are third-line options for allergic rhinitis

A

Cetirizine, fexofenadine, and loratadine are 2nd gen antihistamines which are third-line options for allergic rhinitis
* Minimal effect on congestion

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21
Q

[Drug class] should not be used for more than 3 days in a row due to risk of rebound vasodilation, “rhinitis medicamentosa”

A

Intranasal alpha-agonists (Oxymetazoline) should not be used for more than 3 days in a row due to risk of rebound vasodilation, “rhinitis medicamentosa”
* Generally avoid

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22
Q

Albuterol is a drug that improves asthma via _

A

Albuterol is a drug that improves asthma via beta-agonism

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23
Q

Allergic contact dermatitis is primarily mediated by [cells]

A

Allergic contact dermatitis is primarily mediated by CD4+ T cells
* Poison ivy reaction is another example

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24
Q

Type 4 hypersensitivity is a [time frame], _ mediated reaction

A

Type 4 hypersensitivity is a delayed, T cell mediated reaction
* Time course 24-72 hours

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25
Q

When CD8+ cells recognize foreign cells they release _ and _ to perforate the cell membrane and induce apoptosis

A

When CD8+ cells recognize foreign cells they release perforin and granzymes to perforate the cell membrane and induce apoptosis

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26
Q

Dendritic cells present antigen that is recognized by CD4+ cells which secrete _ and _ to activate CD8+ cells and macrophages in a type 4 hypersensitivity reaction

A

Dendritic cells present antigen that is recognized by CD4+ cells which secrete IL-2 and IFN-gamma to activate CD8+ cells and macrophages in a type 4 hypersensitivity reaction

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27
Q

_ is the test used for evaluation of contact dermatitis

A

Patch testing is the test used for evaluation of contact dermatitis

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28
Q

SJS/TEN is a severe cutaneous adverse reaction in which skin sloughs off, DRESS stands for _ ; both are Type 4 reactions involving T cells

A

SJS/TEN is a severe cutaneous adverse reaction in which skin sloughs off, DRESS stands for drug rash with eosinophilia and systemic symptoms ; both are Type 4 reactions involving T cells
* SJS: 1-4 weeks
* DRESS: 2-6 weeks

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29
Q

Transfusion reactions are an example of [Type] hypersensitivity

A

Transfusion reactions are an example of Type 2 hypersensitivity

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30
Q

Glomerulonephritis, RA, and SLE are examples of [Type] hypersensitivity reactions

A

Glomerulonephritis, RA, and SLE are examples of Type 3 hypersensitivity reactions

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31
Q

B lymphocytes, aka “humoral immunity” is most important in fighting _ infections

A

B lymphocytes, aka “humoral immunity” is most important in fighting bacterial infections
* Without humoral immunity expect chronic sinusitis and pneumonia

32
Q

T lymphocytes or cell-mediated immunity is most important in fighting _ infections

A

T lymphocytes or cell-mediated immunity is most important in fighting fungal and viral infections
* Particularly important for intracellular pathogens
* Also helps in bacterial infections

33
Q

When it comes to fighting off skin and surface infections, [immune cells] are most important

A

When it comes to fighting off skin and surface infections, granulocytes (eosinophil, basophil, neutrophil) are most important

34
Q

Recurrent _ or _ infections do not indicate immune deficiency; however other recurrent infections might

A

Recurrent UTIs or strep throat infections do not indicate immune deficiency; however other recurrent infections might

35
Q

Warning signs of primary immunodeficiency include:
* More than _ ear infections in a year
* More than _ sinus infections in a year
* More than _ pneumonias in a year

A

Warning signs of primary immunodeficiency include:
* More than 4 ear infections in a year
* More than 2 sinus infections in a year
* More than 2 pneumonias in a year

Really you should have concern if you see infections equal to or greater than these numbers

36
Q

Lack of antibodies will lead to recurrent bacterial infections, especially with _ type bacteria

A

Lack of antibodies will lead to recurrent bacterial infections, especially with encapsulated bacteria
* SHiN: Strep pneumo, H flu, Neisseria meningitidus

37
Q

The most common B-cell deficiency is _

A

The most common B-cell deficiency is IgA deficiency
* Recall that IgA is in the mucosa
* Many cases asymptomatic or presents with sinopulmonary infections or anaphylaxis to blood
* Associated with celiac, GI disorders

38
Q

The most common symptomatic immune deficiency is _

A

The most common symptomatic immune deficiency is common variable immune deficiency (CVID)

39
Q

Symtoms of CVID most commonly present at age _

A

Symtoms of CVID most commonly present at age 20-40
* Presents with recurrent sinopulmonary infection
* Bronchiectasis affects 30-50% of patients

40
Q

CVID is caused by _

A

CVID is caused by faulty B cell differentiation into plasma cells

41
Q

How do you work up a possible B-cell deficiency?

A
42
Q

DiGeorge is caused by chromosome deletion at _ ; it is [inheritance pattern]

A

DiGeorge is caused by chromosome deletion at 22q11 ; it is autosomal dominant

43
Q

DiGeorge syndrome is ultimately caused by a failure of _ development

A

DiGeorge syndrome is ultimately caused by a failure of pharyngeal pouch development, pouches 3, 4
* Pouch 3 –> thymus + inferior parathyroid
* Pouch 4 –> superior parathyroid

44
Q

The downstream effect of absent thymus is _

A

The downstream effect of absent thymus is improper T cell differentiation
* Recall that the T cells go from bone marrow –> thymus to mature
* These patients get recurrent viral infections and CMV, EBV, JC virus, VZV + fungal infections, Candida, PJP, Crypto

45
Q

The mneumonic to remember DiGeorge symptoms is _

A

The mneumonic to remember DiGeorge symptoms is CATCH22
* Cardiac
* Abnormal facies
* Thymic hypoplasia
* Cleft palate
* Hypoparathyroidism
* Chromosome 22q11

46
Q

SCID is often caught in newborns but at least seen by _

A

SCID is often caught in newborns but at least seen by year 2 of life
* Recall that newborns get maternal antibodies for the first 6 months

47
Q

Signs of SCID

A

SCID presents with:
* Recurrent, severe infections (T cell, B cell, NK cell dysfunction)
* Chronic diarrhea, failure to thrive
* Absence of lympoid tissue (tonsils, adenoids, axilla)

48
Q

The most common cause of SCID is a _ defect which is [inheritance pattern]

A

The most common cause of SCID is a IL-2 receptor gamma chain defect which is X-linked recessive

49
Q

The less common, autosomal recessive form of SCID is caused by _

A

The less common, autosomal recessive form of SCID is caused by ADA deficiency
* Build up of metabolic toxins that destroy T and B cells

50
Q

MMR is a [type] vaccine

A

MMR is a live attenuated vaccine

51
Q

Varicella is a [type] vaccine

A

Varicella is a live attenuated vaccine

52
Q

Live attenuated vaccines induce [immune response]

A

Live attenuated vaccines induce humoral IgG and cellular
* MHCI presents pathogen to CD4+
* CD4+ activates B cell and allows class switching –> IgG
* Dendritic cell can be infected and also present via MHCII –> cellular response

53
Q

Whole inactivated vaccines will induce [immune] response

A

Whole inactivated vaccines will induce humoral IgG response
* Cannot infect the dendritic cells to trigger cellular response (CD8+)
* However, can be presented via MHC-I, activate CD4+, class switch

54
Q

Subunit protein inactivated vaccines induce [immune] response

A

Subunit protein inactivated vaccines induce humoral IgG response
* Cannot infect the dendritic cells to trigger cellular response (CD8+)
* However, can be presented via MHC-I, activate CD4+, class switch

55
Q

Subunit polysaccharide inactivated will induce [immune response]

A

Subunit polysaccharide inactivated will induce humoral IgM only
* Its an inactivated vaccine, so it cannot infect dendritic cell = no MHC-II and no cellular response
* MHC I can only present protein so it cannot present it either; no class switching, IgM only

56
Q

Subunit polysaccharide + conjugate inactivated vaccines will induce [immune response]

A

Subunit polysaccharide + conjugate inactivated vaccines will induce humoral IgG
* Because the polysaccharide has been conjugated to a protein we can get class switching

57
Q

The MMR and varicella vaccine can be given to HIV+ patients if CD4+ cell count is at least _

A

The MMR and varicella vaccine can be given to HIV+ patients if CD4+ cell count is at least 200
* Live vaccines are contraindicated in pregnancy and patients with immunodeficiency because they can revert to a virulent form

58
Q

[Coombs test] detects antibodies attached to the RBC surface

A

Direct coombs test detects antibodies directly attached to the RBC surface

59
Q

[Coombs test] detects presence of unbound antibodies in the serum

A

Indirect coombs test detects presence of unbound antibodies in the serum

60
Q

Rheumatoid arthritis is an example of a [hypersensitivity reaction]

A

Rheumatoid arthritis is an example of a type III hypersensitivity

61
Q

[Transfusion rxn] involves urticaria, pruritis, wheezing, hypotension, respiratory arrest within minutes to 3 hours of receiving blood products

A

Allergic/anaphylactic reaction involves urticaria, pruritis, wheezing, hypotension, respiratory arrest within minutes to 3 hours of receiving blood products

62
Q

Fever, hypotension, tachypnea, tachycardia, flank pain, hemoglobinuria, and jaundice within 24 hours of blood transfusion may be [transfusion reaction]

A

Fever, hypotension, tachypnea, tachycardia, flank pain, hemoglobinuria, and jaundice within 24 hours of blood transfusion may be acute hemolytic transfusion reaction

63
Q

Acute hemolytic transfusion reaction is a [type HS reaction]

A

Acute hemolytic transfusion reaction is a type II hypersensitivity
* Which typically causes intravascular hemolysis- hemoglobinuria

64
Q

Acute hemolytic transfusion reactions typically occur secondary to _

A

Acute hemolytic transfusion reactions typically occur secondary to ABO blood group incompatability

65
Q

[Transfusion reaction] occurs when cytokines created by donor WBCs accumulate during storage of blood products

A

Febrile nonhemolytic transfusion reaction occurs when cytokines created by donor WBCs accumulate during storage of blood products

66
Q

Febrile nonhemolytic transfusion reactions will present as _

A

Febrile nonhemolytic transfusion reactions will present as fever, headachem chills, fushing within 1-6 hours
* From preformed cytokines

67
Q

Respiratory distress and noncardiogenic pulmonary edema following a transfusion is likely _

A

Respiratory distress and noncardiogenic pulmonary edema following a transfusion is likely Transfusion related acute lung injury (TRALI)

68
Q

Transfusion related acute lung injury (TRALI) occurs due to _

A

Transfusion related acute lung injury (TRALI) occurs due to neutrophil activation by a product in the blood –> inflammation –> pulmonary edema
* Neutrophils sequestered in pulmonary vasculature get released

69
Q

[Transfusion rxn] is a response to a foreign antigen on donor RBCs that was previously encountered by recipient and presents within 1-2 weeks

A

Delayed hemolytic transfusion reaction is a response to a foreign antigen on donor RBCs that was previously encountered by recipient and presents within 1-2 weeks

70
Q

Thrombosis of graft vessels is most indicative of [transplant rejection]

A

Thrombosis of graft vessels is most indicative of hyperacute transplant rejection
* Pre-existing recipient antibodies

71
Q

Vasculitis of graft vessels is most indicative of [transplant rejection]

A

Vasculitis of graft vessels is most indicative of acute transplant rejection
* Cellular or humoral response

72
Q

[Transplant rejection] is dominated by arteriosclerosis

A

Chronic transplant rejection is dominated by arteriosclerosis

73
Q

Graft-vs-host disease is caused by _

A

Graft-vs-host disease is caused by grafted immunocompetent T cells proliferating in the host and targeting host

74
Q

Name (3) B cell disorders and what patients most often present with

A
  1. Bruton agammaglobulinemia: defect in BTK results in no B-cell maturation –> recurrent bacterial and enteroviral infection after 6 months of age
  2. Selective IgA deficiency mostly asymptomatic; GI infections, atopy/anaphylaxis/autoimmune
  3. Common variable immunodeficiency defect in B-cell differentiation –> sinopulmonary infections, increased risk of autoimmune
75
Q

_ is a deficiency of Th17 cells due to STAT3 mutation which impairs the recruitment of neutrophils to sites of infection

A

Autosommal dominant hyper-IgE syndrome is a deficiency of Th17 cells due to STAT3 mutation which impairs the recruitment of neutrophils to sites of infection
* Also called Job syndrome

76
Q

Failure to thrive, chronic diarrhea, thrush, and recurrent infections (all kinds) is a common presentation of _

A

Failure to thrive, chronic diarrhea, thrush, and recurrent infections (all kinds) is a common presentation of SCID
* Also decreased TRECs, absent thymic shadow and germinal centers

77
Q

Hyper IgM syndrome is most commonly due to defective _

A

Hyper IgM syndrome is most commonly due to defective CD40L on Th cells
* Will present with severe pyogenic infections early in life