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Royal College Exam > Allergy & Immunology > Flashcards

Allergy & Immunology Flashcards

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1
Q

What is an important organism to vaccinate against in children with Nephrotic syndrome?

A

Strep pneumo (pneumococcal polysaccharide 23-valent vaccine is recommended)

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2
Q

What is the risk associated with the oral polio vaccine?

A

Vaccine viruses are excreted in the stool of the vaccinated person for up to 6 weeks after a dose.
Maximum viral shedding occurs in the first 1–2 weeks after vaccination, particularly after the first dose.
Vaccine viruses may spread from the recipient to contacts. Persons coming in contact with fecal material of a vaccinated person may be exposed and infected with vaccine virus.

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3
Q

What are the CPS recommendations for post-splenectomy antibiotic prophylaxis?

A

Antibiotic prophylaxis for a minimum of two years postsplenectomy and for all children <5 years of age. Lifelong prophylaxis in all cases is ideal!

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4
Q

What is the percent cross-reactivity between penicillins and cephalosporins?

A

2% (risk of allergic reaction to cephalosporins in patients with positive skin test responses to penicillin)

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5
Q

What are key features of Wiskott-Aldrich syndrome?

A

Eczema, thrombocytopenia, recurrent infections, low IgM, high IgE and IgA

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6
Q

Diagnostic criteria for anaphylaxis

A

Any one of the following three criteria:

  • Acute onset of an illness with involvement of the skin and/or mucosal tissue AND at least one of respiratory compromise or hypotension
  • Two or more systems involved (skin/mucosa, resp, CV, GI) after exposure to likely allergen
  • Reduced BP following exposure to known allergen (>30% drop)
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7
Q

Child with abscesses, lymphadenopathy, serratia infection

A

Chronic granulomatous disease

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8
Q

Unilateral facial weakness, vesicles in ear canal

A

Ramsay-Hunt Syndrome (herpes zoster oticus) - treated with acyclovir and steroids

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9
Q

Treatment of type III hypersensitivity reaction (ie serum sickness)

A

anti-histamine and NSAIDs

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10
Q

How do you diagnose CGD?

A

nitroblue tetrazolium test for neutrophil oxidative ability

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11
Q

Influenza vaccine and egg allergy - contraindicated?

A

Administration of inactivated trivalent or quadrivalent influenza vaccines is safe for individuals with egg allergy. Unless children have experienced an anaphylactic reaction to a previous dose of influenza vaccine, they can and should be immunized with a full dose of trivalent or quadrivalent inactivated vaccine. Don’t give the live attenuated vaccine (not tested).

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12
Q

Low IgA, normal IgM and IgG

A 
IgA deficiency 
Can’t be diagnosed until 4 years of age (when IgA reaches adult levels). Occurs in 1/500, most asymptomatic. Associated with recurrent sinopulmonary infections, IgG2 subclass deficiency, food allergy, autoimmune disease and celiac.
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13
Q

What is important to test in a patient with neisseria meningitis?

A

Terminal complement (C5-9) - the membrane attack complex

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14
Q

What patients respond to allergen immunotherapy?

A

seasonal/perennial allergic rhinoconjunctivitis, asthma triggered by allergen exposures, and insect venom sensitivity

(no evidence for food allergy, atopic dermatitis, latex allergy, and acute/chronic urticaria)

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15
Q

What can result in false positive RAST testing?

A

High IgE

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16
Q

Infections, absent thymus?

A

SCID

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17
Q

Hypocalcemia, congenital heart disease, absent thymus?

A

DiGeorge (22q11)

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18
Q

SCID genetic defect

A

x-linked, gamma chain defect of the IL-2 receptor

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19
Q

Hallmark of leukocyte adhesion deficiency

A

delayed separation of umbilical cord

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20
Q

Classic presentation of SCID

A

thrush, recurrent sepsis, pneumonia, otitis media, diarrhea, FTT, absent lymphoid tissue

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21
Q

Work up of suspected humoral immunity deficiency

A
  • CBC, diff
  • immunoglobulins
  • vaccine titres
  • RBC isohemagglutinin (IgM)
  • CXR (to evaluate for thymus, bronchiectasis)
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22
Q

What is a hallmark sign of CVID?

A

Recurrent sino-pulmonary infections

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23
Q

IVIG replacement therapy dose

A

400-600 mg/kg Q3-4 weeks

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24
Q

In what condition could you have anaphylaxis to IVIG?

A

IgA deficiency

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25
Q

T-cell problem work-up

A
  • CBC & diff
  • intradermal candida skin testing
  • T & B cell markers (flow cytometry)
  • Day 3 proliferation (mitogen - stimulate proliferation)
  • Day 6 proliferation (antigen)
  • thymic biopsy
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26
Q

Most common cardiac issue in DiGeorge?

A

interrupted aortic arch, type B

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27
Q

Can SCID babies be breastfed?

A

NO. Unless the milk is irradiated.

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28
Q

Features of Omenn’s syndrome

A

Erythroderma
Lymphadenopathy
Hepatosplenomegaly
Short limbs

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29
Q

Ataxia telangiectasia

A

Abnormal DNA repair
15% develop malignancy
up to 80% have IgA deficiency

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30
Q

Wiskott-Aldrich Syndrome - immunoglobulin pattern

A

normal IgG, low IgM and elevated IgA and E

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31
Q

Wiskott-Aldrich syndrome clinical symptoms

A

x-linked, eczema, thrombocytopenia and recurrent pyogenic infections

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32
Q

What prophylaxis should a patient with CGD be on?

A

septra, itraconazole

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33
Q

What component of penicillin are people anaphylactic to?

A

Minor component

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34
Q

What component of penicillin are people allergic to?

A

Major component

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35
Q

How often do IgE-mediated systemic reactions occur to penicillin?

A

approximately 2% of the time

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36
Q

Cross-reactivity of penicillins and cephalosporins?

A

<2%

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37
Q

most common food allergy in kids?

A

cows milk

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38
Q

what percent of children will outgrow peanut allergy?

A

20%

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39
Q

When should you evaluate for an immunodeficiency?

A
  • unusual bugs (PJP, aspergillus, etc)
  • unusual site (CNS, liver)
  • 2 or more serious resp/bacterial infections within 1 yr (pneumonia, cellulitis, etc)
  • 1 or more systemic bacterial infection (sepsis)
  • usual bugs, but very severe
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40
Q

Hypocalcemia
Unusual facies/ears
Cardiac disease

A

DiGeorge (22q11)

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41
Q

Delayed separation of the umbilical cord
Leukocytosis
Recurrent infections

A

Leukocyte adhesion defect

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42
Q

Persistent thrush, diarrhea
FTT
Pneumonias

A

SCID

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43
Q

Bloody stools
draining ears
atopic eczema
thrombocytopenia

A

Wiskott-Aldrich syndrome

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44
Q

CBC findings in SCID

A

lymphopenia (t-cells = 70% of lymphocytes)

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45
Q

Suspected B-cell defect. What tests would you order to work-up?

A

CBC, diff, ESR
IgA, IgM, IgG
Vaccine antibody titres
Isohemagglutinins

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46
Q

Suspected T-cell defect. What tests could you do to evaluate?

A

CBC (lymphopenia)

Candida albicans intradermal skin test

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47
Q

Screen for phagocytic cell defects

A

Absolute neutrophil count

Respiratory burst assay

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48
Q

Screen for complement deficiency

A

CH50

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49
Q

Most common B-cell defect

A

IgA deficiency

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50
Q

Three conditions where isohemagglutinins would be low/absent

A

B cell defect
First 2 yrs of life
Blood type AB

51
Q

If you wanted to test antibody response to a polysaccharide vaccine, which could you use?

A

PPV23 (pneumococcal polysaccharide vaccine)

52
Q

If you wanted to test antibody response to a protein antigen, which vaccine could you use?

A

DTaP

53
Q

What should you do if IgM/IgG are low in serum, but the patient has normal antibody responses to vaccines?

A

Check for protein loss in gut (enteropathy) or urinary tract (nephrotic syndrome)

54
Q

If a child lacks IgG2, what will they not be able to do?

A

make antibodies in response to polysaccharide antigens

55
Q

If you detect agammaglobulinemia, what should you test next?

A

flow cytometry for b-cells

absent in x-linked agammaglobulinemia, present in CVID

56
Q

B cell specific CD antigens

A

CD 19

CD 20

57
Q

Negative candida skin test, what does this mean?

negative = < 10mm at 48 hrs

A

Normal T-cell function!

58
Q

T cell specific CD antigens

A

CD2, CD3, CD4, CD8

59
Q

Child with lymphopenia. What type of cell defect should you suspect?

A

T-cell! Usually 70% of lymphocytes are T-cells

60
Q

You suspect a T-cell deficiency, but the T-cell numbers are normal on flow cytometry. What do you do next?

A

Mitogen stimulation testing

functional testing of T-cell

61
Q

Recurrent staph abscesses + infections with gram-negative bugs. What type of defect?

A

Phagocytic cells

62
Q

How do you assess for a phagocytic cell defect?

A

Check neutrophil respiratory burst

Best test = flow cytometric assessment of the respiratory burst using rhodamine dye

(replaced nitroblue tetrazolium dye test)

63
Q

Inheritance of CGD

A

1 type is x-linked (65%)

3 types are AR (35%)

64
Q

Most common cause of an abnormal CH50

A

Delay or improper transfer of the specimen to the lab

65
Q

Newborns are deficient in IgM. Why is this? What does this make them susceptible to?

A

Only IgG crosses the placenta

They have increased susceptibility to gram-negative organisms

66
Q

When does the thymus begin to involute?

A

after puberty

67
Q

No tonsils/lymph nodes, flow cytometry shows no circulating B-cells, very low (almost absent) IgG/M/A/E
normal thymus and increased number of T-cells

A

X-linked agammaglobulinemia

Due to mutation in Bruton tyrosine kinase gene

68
Q

What kind of infections do boys with X-linked agammaglobulinemia get?

A

Recurrent sinopulmonary infections with respiratory pathogens (strep, h.flue, staph, pseudomonas, mycoplasma)
ALSO
hepatitis, enterovirus

69
Q

What other cell line can be affected in XLA?

A

they can have neutropenia

BTK also important for myeloid cell differentiation

70
Q

Treatment of XLA?

A

Replace absent immunoglobulins! (IVIG)

71
Q

What would flow cytometry and mitogen stimulation tests (of B-cells) show in CVID?

A

Normal numbers of circulating peripheral B-cells, no antibody response to mitogens (or T-cells)

72
Q

Patient with CVID, increased risk of what cancer?

A

Lymphoma! (438x increased risk)

73
Q

Encapsulated bacteria

A 
SHiNE SKiS
Strep pneumo
Haemophilus influenza typeB
Neisseria meningitidis
E.coli

Salmonella
Klebsiella
Group B Strep

74
Q

Medications that may be related to CVID or IgA deficiency

A

Phenytoin
Gold
Sulfasalazine
Penicillamine

75
Q

What GI infection do with IgA deficiency get?

A

Giardia

76
Q

If someone had anaphylaxis after IVIG or blood products, what would you suspect?

A

IgA deficiency

44% of IgA deficient people have antibodies to IgA - if these are IgE antibodies, can cause anaphylaxis!

77
Q

Is hyper IgM syndrome of T-cell or B-cell defects?

A

T-cell defect! B-cells function normally, but t-cells can’t undergo gene rearrangement to direct B-cells to make other types of immunoglobulins

78
Q

Features of hyper IgM syndrome

A 
Profound neutropenia
PJP pneumonia
Recurrent infections with encapsulated organisms (can't make IgG)
Warts
Crytosporidium enteritis
Liver disease
Increased risk of malignancy
79
Q

Baby with DiGeorge syndrome. What condition did mom have during pregnancy?

A

15% of babies with DiGeorge are born to diabetic mothers

80
Q

Which antibody deficiency is at increased risk of PJP pneumonia?

A

Hyper IgM Syndrome - is actually T-cell defect, unable to “switch” to stimulate B-cells to make other types of immunoglobulins

81
Q

What conditions would prompt you to look for DiGeorge (and for T-cell deficiency)

A

primary hypoparathyroidism, CHARGE syndrome, truncus arteriosus, and interrupted aortic arch type B

82
Q

Chronic mucocutaneous candidiasis + hypoparathyroidism (hypocalcemia). What should you check for?

A

test for addison’s disease

could be autoimmune polyendocrinopathy type I, or APECED

83
Q

Profound lymphopenia, no T cells, absent antibody response, rachitic rosary.

A

ADA-deficient SCID

(presence of rib cage abnormalities similar to a rachitic rosary and numerous skeletal abnormalities of chondro-osseous dysplasia)

84
Q

Omenn syndrome

A

AR, fatal

Erythroderma, profound diarrhea / FTT, lymphadenopathy, HSM, eosinophilia

Absence of BOTH B+T cells

85
Q

Immunoglobulin pattern in Wiskott-Aldrich

A

Low IgM

High IgE/IgA

86
Q

Clinical features of Wiskott-Aldrich

A

Thrombocytopenia (microplatelets)
Eczema
Recurrent infections (encapsulated organisms, herpes viruses, PJP)

87
Q

Major causes of death (name 3) in Wiskott-Aldrich syndrome

A

hemorrhage
Recurrent infection
EBV-associated malignancy

88
Q

Management of Wiskott-Aldrich

A

Platelet transfusions prn
IVIG
BMT

89
Q

What should you avoid in a patient with Ataxia-Telangectasia Syndrome?

A

X-rays!

DNA repair defect, increased risk of malignancy

90
Q

Recurrent staph infections, candida, retained primary teeth, coarse facial features. What syndrome?

A

Hyper IgE

91
Q

Treatment of hyper IgE syndrome

A

anti-staph antibiotics

IVIG replacement

92
Q

Causes of neutropenia

A 
Infectious
Post-infectious
Medication induced
Starvation/anorexia
Ineffective myelopoesis
Syndrome-associated
93
Q

Severe congenital neutropenia - other blood counts?

A

Monocytosis, eosinophilia

Often anemia of chronic disease

94
Q

Risk of what cancer with severe congenital neutropenia?

A

AML

MDS

95
Q

Pancreatic insufficiency, neutropenia

A

Shwachman-Diamond

96
Q

Schwachman-Diamond
Inheritance?
Major features?

A

AR mutation in SBDS gene
Neutropenia
Pancreatic insufficiency
FTT

97
Q

Delayed separation of the umbilical cord - think of what condition?

A

Leukocyte adhesion defects

98
Q

Neutrophil count in leukocyte adhesion defects?

A

Often > 30! very high peripheral blood neutrophilia, neutrophils cannot extravasate and migrate

99
Q

Diagnosis of leukocyte adhesion defect (type 1)

A

flow cytometry - absence of CD11b/CD18 on neutrophils

100
Q

Diagnosis of Chediak-Higashi syndrome

A

Large lysosomal inclusions in all nucleated cells - may not be evident on peripheral blood (large cells can’t get out of bone marrow) - check BMA if suspect CHS!

101
Q

Treatment of chediak-higashi syndrome?

A

High dose Vit C

BMT

102
Q

Inheritance of CGD

A

1 type x-linked

3 types AR

103
Q

Most common organism causing infection in CGD

A

Staph aureus

104
Q

Non-immune signs of CGD

A 
anemia of chronic disease
FTT
HSM
lymphadenopathy
dermatitis
ginigivitis
hydronephrosis
pyloric outlet narrowing?
105
Q

Supportive care in CGD

A

septra prophy
itraconazole prophy
interferon gamma 3x/wk (mechanism unknown)

106
Q

What order do classical complement proteins fix to the immune complex?

A

1-4-2-3

107
Q

Terminal complement deficiency (C5-8) predisposes you to what types of infections?

A
  • meningococcal meningitis

- extragenital gonococcal infection

108
Q

Suppurative lymphadenopathy is a feature of what condition?

A

CGD

109
Q

Which condition is associated with a lack of purulent discharge?

A

LAD

110
Q

Which condition is prone to getting serratia?

A

CGD

111
Q

Sinopulmonary infections + enterovirus

A

XLA

112
Q

most common cause of out-of-hospital anaphylaxis

A

food allergy (most commonly, peanut)

113
Q

Common causes of anaphylaxis in children

A 
Food (peanuts, milk, eggs, fish)
Drugs (Penicillins)
Insect venom (bee)
Latex
Vaccines
114
Q

Dose and administration of epinephrine for anaphylaxis

A

0.01 mg/kg IM (up to 0.3)

If prescribing EpiPen:

  1. 15mg (for 8-25 kg)
  2. 3mg (for >25 kg)
115
Q

Other medications used in anaphylaxis (other than epi)

A

Corticosteroids (Methylpred IV)

H1 blockers (Cetirizine/Diphenhydramine)

H2 blockers (Ranitidine/Cimetidine)

O2

IV fluids

Salbutamol (beta-agonist)

116
Q

Kid treated with amoxicillin and develops a non-pruritic rash over body. What infection did they likely have?

A

EBV

117
Q

Two antibiotic classes with cross-reactivity to penicillins

A

Cephalosporins

Carbapenems

118
Q

Most common hematologic abnormality in allergic patients

A

Eosinophilia

119
Q

Name two diseases that have significantly elevated IgE

A

Hyper IgE

Allergic bronchopulmonary aspergillosis

120
Q

What is Red Man syndrome and how do you manage it?

A

Non-specific histamine release due to Vancomycin.

Can be prevented by slowing infusion rate and administering H1 blocker (benadryl)

121
Q

What autoimmune antibody is associated with chronic urticaria?

A

anti-thyroid antibodies

122
Q

patient with chronic urticaria. what might you see on skin testing?

A

positive autologous skin test (ASST) result (their own serum injected intradermally)

123
Q

How do you treat chronic urticaria?

A

H1 antihistamine
if not responding - increase dose
if still not responding, add H2 blocker (NOT H2 alone)
consider short course of steroids

124
Q

What is elevated in the blood in anaphylaxis?

A

plasma b-tryptase

Royal College Exam (32 decks)

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