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Board Review > Allergy and Immunology > Flashcards

Allergy and Immunology Flashcards

1
Q

treatment of wiskott-aldrich?

A

bone marrow transplant, or splenectomy

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1
Q

besides infections, what other findings can you have with CVID?

A

non-caseating granulomas in liver, spleen, lungs (can have hepatosplenomegaly); celiac-type sprue, polyarthritis, other autoimmune conditions, increased risk of lymphoma, increased risk of giardia infections

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1
Q

how do you treat cyclic neutropenia?

A

GCSF (autosomal dominant)

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2
Q

what other conditions are associated with Purine Nucleoside Phosphorylase deficiency SCID?

A

neurologic disorders, autoimmune hemolytic anemia, and thrombocytopenia

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3
Q

what type of vaccines should be avoided in children with SCID?

A

live-attenuated viruses?

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3
Q

what groups of people MUST be desensitized to penicillin for appropriate treatment?

A

pregnant women with syphillis, or person with neurosyphillis

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4
Q

what type of cancers are common with ataxia-telangeiectasia?

A

lymphoma or lymphocytic leukemias

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5
Q

what gene is associated with wiskott-aldrich?

A

Xp11.22

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5
Q

what are some examples of type 4 reactions

A

tuberculin sensitivity, some contact dermatitis (cell-mediated hypersensitvities - previously sensitized T-cells)

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6
Q

B-cell deficiencies cause what general type of infections?

A

recurrent sinopulmonary

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6
Q

what complement deficiency should you look for in someone with meningococcal meningitis?

A

C5-C9 deficiency

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7
Q

what type of mastocytosis does not have mast cells in the skin?

A

malignant: causes severe systemic symptoms, hepatosplenomegaly, lymphadenopathy

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8
Q

what type of blood should babies with SCID receives?

A

irradiated (to prevent GVHD from T-cell transfusion)

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8
Q

how long must children be off antihistamines before skin testing?

A

at least 72 hours, preferably longer

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9
Q

how do you diagnos chronic granulomatous disease?

A

nitroblue tetrazolium (NBT) test or dihydrorhodamine (DHR) oxidation (flow cytometry)

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9
Q

should children with HIV get MMR and varicella vaccines

A

yes! (no oral polio though)

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10
Q

what are some of the primary B-cell immunodeficiencies?

A

X-linked agamma/hypogammaglobulinemia, CVID, hyper-IgM

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10
Q

how do you treat hyper IgM?

A

IVIG and Bactrim (PCP prophylaxis)

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11
Q

what are the levels of T- B- and NK cells in ADA SCID?

A

NO t-, b- or NK cells

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12
Q

treatment for SCID?

A

bone-marrow transplant (to make Tcells) AND IVIG (to replace antibodies cause B-cell function is usually not restored)

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12
Q

by what age do children with transient hypogammaglobulinemia reach normal IgG levels?

A

3-4 years (only tx with IVIG before then if having recurrent severe infections)

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13
Q

what are first line tests for T-cell disorders?

A

flow cytometry and delayed type hypersensitivity (candida skin testing)

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15
Q

what other abnormalities are common with ADA SCID?

A

skeletal (cupping and flaring of costochondral junctions, pelvic dysplasia)

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15
Q

what are some signs and symptoms of serum sickness?

A

fever, skin rash, joint pain, lymphadenopathy, myalgias, proteinuria, itching, redness, urticaria, angioedema, nausea, vomiting

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16
Q

what problems does Job syndrome cause?

A

eczema, scoliosis, delayed dental exfoliation, fractures, recurrent skin infections/abscesses with strep pneumo, staph and h flu

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17
Q

can household contacts of children who are immunocompromised receive live vaccines?

A

yes they can receive MMR and varicella - they CANNOT receive oral polio and should not get flumist

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18
Q

what is the presentation for x-linked agammaglobulinemia?

A

boys around 1 yr of life with recurrent penumonia, otitis, sinusitis - small lymph nodes and tonsils

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19
Q

what are children with immunodeficiency at higher risk of?

A

malignancy

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19
Q

what antibiotics commonly cause serum sickness?

A

cefaclor and penicillin

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20
Q

complement deficiencies tend to cause what?

A

overwhelming sepsis

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20
Q

findings in ataxia-telangeiectasia?

A

cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, high incidence of cancer, high sensitivity to ionizing radiation

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21
Q

what vaccines should be avoided in children on chemo/receiving BMT or with immunodeficiencies?

A

BCG, oral polio, MMR, varicella

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22
Q

what class of medicines is relatively contraindicated in those with severe anaphylactic allergies?

A

beta-blockers (can dampen response to epinephrine)

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24
Q

what immunodeficiencies are both cellular and humoral deficiencies?

A

SCID, wiskott-aldrich, ataxia-telangeiectasia, bloom syndrome, and nimegen breakage syndrome

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24
Q

what physical findings are seen in Job syndrome?

A

asymmetric face, broad nose, triangular jaw, prominent forehead

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25
Q

when do Type 1 hypersenstivity reactions usually start?

A

within one hour of exposure

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27
Q

what are clinical findings associated with wiskott-aldrich?

A

bleeding (esp after circ); staph infections, recurrent sinopulmonary infections, small platelets

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27
Q

how does ataxia-telangeiectasia present?

A

ataxia usually by age 5, then the telangiectasias form in bulbar conjunctiva, ears, neck, and elbows

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28
Q

infants with “complete” digeorge are at risk of what?

A

GVHD with blood transfusions (like SCID) - have NO t-cells and low b-cell fxn (most have “incomplete” with some t-cell and normal b-cell function)

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28
Q

what virus is x-linked lymphoproliferative disease (duncan syndrome) associated with?

A

EBV - can cause fulminant hepatitis, lymphoma, agranulocytosis, aplastic anemia,

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29
Q

what are the levels of T- B- and NK cells in x-linked SCID?

A

NO t-cells or NK cells. Some B cells but they do not function normally.

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30
Q

does a negative skin test indicated NO allergy?

A

yes

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31
Q

T-cell deficiencies cause what general type of infections?

A

opportunisitic

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32
Q

does the bone marrow produce normal B cells in X-linked agammaglobulinemia?

A

yes it makes normal precursors, but the cells do not mature properly, so not functional

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33
Q

what vaccine do you NOT give boys with x-linked agammaglobulinemia?

A

oral polio vaccine

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34
Q

how is ADA SCID transmitted?

A

autosomal recessive

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35
Q

when does the late phase of type 1 reactions start?

A

3-12 hours later, lasts hours to days

37
Q

What metabolic form of SCID is most common?

A

adenosine deaminase deficiency (ADA) - 15-20% of SCID cases

37
Q

what types of infections are seen with leukocyte adhesion defects?

A

recurrent necrotizing infections where body meets the environment (skin, mucosa, gut, lungs)

38
Q

if a child presents with abdominal pain AND extremity swelling, what should you think of?

A

hereditary angioedema (can try and treat with e-aminocaproic acid or epinephrine)

39
Q

how do you treat CGD?

A

BMT or prophylactic antibiotics/antifungals and interferon gamma if no match

40
Q

which syndrome is a primary T-cell immunodeficiency?

A

DiGeorge

41
Q

when you see a vasculitis, what type of hypersensitivity reaction should you think of?

A

type 3 (immune complex reactions)

43
Q

what are the genes in Autosomal Recessive SCID associated with?

A

jak 3 kinase

44
Q

what are first line tests for B-cell disorders?

A

quantitative immunoglobulins, response to vaccines

46
Q

what is the most common form of SCID?

A

x-linked (50% of cases); only affects boys

46
Q

what lab finding is associated with LAD?

A

leukocytosis (since cells can’t leave the bloodstream)

47
Q

what physical findings is often associated with LAD?

A

delayed umbilical cord seperation (also have cigarette paper scarring)

48
Q

what post-pneumonia finding is often seen with Job syndrome?

A

pneumatoceles

48
Q

what causes the type 1 late reactions?

A

cytokine release triggered by the mast cells, leads to eosinophilic infiltrate

50
Q

some causes of pneumonia in SCID?

A

CMV, RSV, PCP

52
Q

what is the mnemonic for wiskott-aldrich?

A

EXIT : eczema, x-linked, immunodeficiency, thrombocytopenia

52
Q

how is CGD usually inherited?

A

x-linked

54
Q

what lab findings are found with ataxia-telangeiectasia?

A

increased alpha1-fetoprotein (AFP), increased CEA, low IgA and IgG possible

56
Q

how do you treat X-linked agammaglobulinemia?

A

IVIG monthly

58
Q

how do you test for LAD?

A

flow cytometry for CD18

59
Q

can you develop serum sickness on a first exposure to a drug?

A

yes

61
Q

what type of infections are seen in hyper IgM?

A

sinopulmonary infections, giardiasis, bacterial and viral meningitis

63
Q

what chromosome is the gene for ADA on?

A

chromosome 20

64
Q

what disease has recurrent sinopulmonary and cutaneous infections, partial oculocutaneous albinism, mild MR, and peripheral neuropathy?

A

Chediak-Higashi syndrome

65
Q

does a positive RAST test or positive skin test indicated allergy?

A

no, only suggest

67
Q

what lab finding is diagnostic for wiskott-aldrich?

A

small platelets

68
Q

what are first line tests for phagocytic disorders?

A

CBC with diff and neutrophil oxidation (either NBT or DHR tests)

69
Q

what are the primary findings in DiGeorge?

A

craniofacial abnormalities, heart defects, absent parathyroids, subclinical hypoparathyroidism, mental retardation is common

71
Q

what should be the first screening tests for a complement deficiency?

A

CH50 (will be low) and C3/C4 levels

72
Q

what type of infections do phagocytic disorders usually cause?

A

skin and organ abscesses

73
Q

what is the first best measure to fight dust mite allergies?

A

zippered pillow and mattress covers, launder bed linens every other week ; could also reduce humidity

75
Q

what is underlying cause of ataxia-telangeiectasia?

A

defect in ATM gene - leads to increased apoptosis and cell death

77
Q

what is bloom syndrome?

A

small stature, teangiectasia, CNS abnormalities, immunodeficiencies, and increased leukemia risk

78
Q

what are the lab findings with x-linked agammaglobulinemia?

A

severe deficiency of all immunoglobulins, no B-lymphocytes in blood or lymph tissue (but can find immature precursor cells)

79
Q

how is hereditary angioedema inherited?

A

autosomal dominant (AD)

80
Q

what causes hereditary angioedema?

A

C1 inhibitor deficiency

81
Q

what lab finding is associated with Chediak-Higashi syndrome?

A

large neutrophil granules (fusion of primary and secondary granules)

82
Q

what are type 2 hypersensitvity (cytotoxic) reactions?

A

autoantibodies bind to target cell receptors or fixed tissue antigens (SLE-related thrombocytopenia, leukopenia, autoimmune hemolytic anemia; also Goodpasture’s and myastenia gravis)

84
Q

how do babies with SCID present?

A

eczematous skin changes, absent thymus, low white count, lung infections, diarrhea, failure to thrive

85
Q

what other lab finding will you seen with hereditary angioedema?

A

low C2 and C4 levels (constantly being used up since there is no inhibition of C1)

86
Q

how is hyper IgM inherited?

A

x-linked (poorer prognosis) or autosomal recessive

87
Q

how do you treat serum sickness

A

usually supportive care (self-limited)

88
Q

what types of infections do children with CVID have?

A

recurrent sinus and pulmonary (encapsulated bacteria)

89
Q

what infections are common with ataxia-telangeiectasia?

A

sinopulmonary infections, bronchiectasis

90
Q

what other diseases are people with C3 deficiency at risk for?

A

SLE, vasculitis, autoimmune diseases

91
Q

how do children with ADA SCID present?

A

within months of birth with profound lympopenia, hypogammaglobulinemia, and recurrent infections (chronic candidasis, PCP pneumonia, CMV< EBV, varicella, severe enterovirus)

92
Q

what causes the type 1 acute reaction?

A

mast cell degranulation leading to histamine release

93
Q

what is hyper IgM syndrome?

A

deficiency of IgG and IgA but increased levels of IgM

94
Q

how is the x-linked type different?

A

also some T-cell dysfunction (problems with CD40) - can present with PCP pneumonia

95
Q

what is CVID?

A

deficiency of at least two classes of immunoglobulins or poor immunoglobulin function (no response to vaccination)

97
Q

what gene is associated with X-linked (Bruton’s) agammaglobulinemia?

A

Xq22 - codes for bruton’s tyrosine kinase which is important for B cell maturation

98
Q

what lab finding is often associated with Job syndrome?

A

elevated IgE

99
Q

what are some examples of type 3 reactions?

A

vasculitis, serum sickness (from exposure to drugs), Ig autoimmune diseases (SLE, hashimoto’s, RA)

100
Q

early infections in SCID?

A

chronic cutaneous candidasis, rotavirus, pneumonia

101
Q

how does CGD present?

A

chronic and recurrent organ and skin abscesses

102
Q

what is urticaria pigmentosa?

A

formation of a wheal on gentle stroking of the skin (darier sign)

103
Q

what is the triad for Wiskott-Aldrich syndrome

A

eczema, thrombocytopenia, susceptibility to bacterial (encapsulated) and opportunisitic infections

104
Q
A

serum sickness

105
Q
A

allergic shiners

106
Q
A

allergic rhinitis

107
Q
A

chronic steroid use: buffalo hump, obesity, moon facies

108
Q
A

vascular ring

109
Q
A

urticaria

110
Q
A

angioedema

111
Q
A

DiGeorge: micrognathia, hypertelorism, low-set malformed ears, midline scar (from surgery), smooth philtrum

112
Q
A

eczema from wiskott-aldrich: note diffuse locations

113
Q
A

pneumatocele: can be associated with staph aureus pneumonias and hyper IgE syndrome (Job syndrome)

114
Q
A

periumbilical infection: think of leukocyte adhesion deficiency (esp if high WBC)

115
Q
A
116
Q

what are the three main types of phagocytic disorders?

A

neutropenia, phagocyte chemotaxis disorders (leukocyte adhesion defects), and phagocyte killing disorders (Job syndrome, CGD, Chediak-Higashi)

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