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Allergy/Immunology Flashcards

1
Q

Name 4 functions of T cells

A

Delayed type hypersensitivity (type IV)

Cytotoxic host-defense functions

Immunoregulatory functions

Respond to MHC class I and class II APCs

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2
Q

What diseases can be associated with recurrent candida infection?

A

Diabetes mellitus
HIV infection
Patients treated with systemic or inhaled glucocorticoids Prolonged courses of antibiotics
CMCC

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3
Q

What is chronic mucocutaneous candidiasis?

A
  1. Recurrent Candida
    Persistent infections of the skin, nails and mucous membranes with candida species
    Disseminated infection RARE
  2. Autoimmunity
    - Cytopenias
  3. Endocrinopathies
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4
Q

What endocrine abnormalities can be associated with CMC?

A 
Hypoparathyroidism
Hypothyroidism
Addison's
T1DM
Gonadal dysfunction
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5
Q

What is the most common immunodeficiency causing recurrent meningococcal meningitis?

A

Late complement deficiency (C5, C6, C7, C8)

Susceptible to Neisseria meningococcal meningitis or extragenital gonococcal infection

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6
Q

What do C1-4 deficiencies typically present with?

A

Rheumatologic/lupus-like disorders

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7
Q

What is the most common complement deficiency?

A

C2

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8
Q

What diseases/infections are associated with C2 deficiency?

A 
SLE-like presentation
Recurrent pyogenic infections with encapsulated bacteria
HSP
RA
Dermatomyositis
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9
Q

What are the clinical features of Wiskott-Aldrich syndrome?

A

Eczema

Thrombocytopenia

Sinopulmonary infections, sepsis/meningitis, PJP, herpes

Watery diarrhea (can be bloody) and petechiae in the first few months of life

Impaired ability to make specific antibodies especially to polysaccharide antigen

Later manifestations: Autoimmunity, EBV-associated malignancies

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10
Q

What laboratory abnormalities do you see in Wiskott-Aldrich?

A

Thrombocytopenia
Low to normal IgG and IgM and high IgA and IgE
Impaired B-cell response to polysaccharide antigens

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11
Q

How is Wiskott-Aldrich inherited?

A

X-linked

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12
Q

What is the most cost effective screening test for T-cell defect?

A

Candida skin test

Intradermal skin testing with candida to evaluate delayed hypersensitivity

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13
Q

When does severe combined immunodeficiency typically present?

A

4-12 months of age, but can present earlier

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14
Q

What are the clinical features of SCID?

A 
Oral Thrush
Persistent/recurrent bacterial, fungal, viral infections
Chronic diarrhea
Pneumonitis
PCP infection
Disseminated CMV
FTT
Small thymus
Absent lymphoid tissue (except Omenn’s)
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15
Q

Name 2 SCID diseases

A

ADA deficiency (defect in purine metabolism)

Omenn’s syndrome (skin rash, HSM, short limbs, lymphadenopathy, eosinophilia, fever)

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16
Q

How do you treat SCID?

A

BMT

Peg-ADA

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17
Q

What is the immunologic abnormality in CVID?

A

Inability of B cells to differentiate into plasma cells capable of secreting Ig

Hypogammaglobulinemia

Normal number of B cells

Poor or absent response to immunizations (despite having normal number of B-cells)

Normal or near normal cellular immunity

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18
Q

What are the clinical features of CVID?

A

Recurrent Infections:

  • Sinopulmonary and chronic GI infections
  • H.influenzae, streptococcus pneumoniae, staph
  • Mycoplasma, enteroviruses, Giardia

Respiratory:

  • Bronchiectasis
  • Pulmonary fibrosis with granulomas

Allergic disease:
-Food allergy, eczema, urticaria, rhinitis

Gastrointestinal:

  • IBD picture - Diarrhea, weight loss, malabsorption
  • Giardia
  • Nodular lymphoid hyperplasia of the small bowel
  • Chronic pangastritis
  • FTT

Autoimmune disease

  • Autoimmune cytopenias
  • Evans syndrome

Granulomatous inflammation
-Lung, liver, spleen, skin

Malignancy

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19
Q

What laboratory features do you see in CVID?

A

Autoimmune cytopenias

Decreased serum immunoglobulins

Specific antibody titres decreased/absent

Impaired vaccine response

T-cell abnormalities in up to 50% of patients
(Reversed CD4/8 ratio, decreased function, restricted repertoire)

Usually normal number of B-cells

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20
Q

What types of infections are seen in X-linked agammaglobulinemia?

A 
Sinopulmonary
GI infection
Sepsis
Septic/non-infectious arthritis
Meningitis
PCP infection
S. pneumonia, Hib, enterovirus
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21
Q

What is the immunologic abnormality in X-linked agammaglobulinemia?

A

Lack of B cells, plasma cells (this is what differentiates it from CVID)
No immunoglobulin
Abnormal lymph tissue
BM shows arrest of B cell maturation

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22
Q

What is the most common mutation in X-linked agammaglobulinemia?

A

90 - 95% of patients with XLA have a BTK mutation

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23
Q

How do you treat X-linked agammaglobulinemia?

A

Ig replacement
Monitor for IgG levels
Aggressive antibiotic treatement of infections
PFTs

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24
Q

What are the clinical features of ataxia telangiectasia?

A

Progressive cerebellar ataxia

  • Earliest manifestation
  • Confined to wheelchair by 10-12 years of age

Telangectasias (eyes, ears)
-Appear at age 3-5

Recurrent sinopulmonary infections

Progressive pulmonary disease (multifactorial)

Increased risk of malignancy (mostly leukemia, lymphoma)

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25
What are the immunologic abnormalities in ataxia telangiectasia?
50% - 80% have absent IgA Reduced ability to make antibodies (esp to polysaccharide antigens) Slightly decreased T-cell numbers (not usually clinically significant) Decreased proliferations
26
What is the most common laboratory abnormality in ataxia telangiectasia?
Absent IgA
27
What 3 features are required to have GVHD?
Histocompatibility differences between the graft donor and recipient Presence of immunocompetent cells in the graft that can respond to host antigens Immunoincompetence of the host, preventing rejection
28
What is the time frame for acute GVHD?
14 (engraftment) to 100 days after transplantation
29
What are the clinical features of acute GVHD?
Skin Liver GI (diarrhea)
30
How do you treat acute GVHD?
Steroids CSA MTX
31
What is the timeframe for chronic GVHD?
3 to 15 months after transplantation and may last for years
32
What are the clinical features of chronic GVHD?
Progressive systemic sclerosis Skin-mucous membranes may become dry Hematologic-anemia thrombocytopenia, eosinophilia GI-esophagus, small intestine (causing weight loss) Liver-cholestatic liver disease Lungs-restrictive pulmonary defects
33
How do you treat chronic GVHD?
Prednisone CSA Biologics
34
What is the inheritance pattern of CGD?
Majority x-linked | Some AR
35
How do you diagnose CGD?
NOBI is test of choice
36
How do you treat CGD?
Aggressive antibiotic therapy (itraconazole and septra prophylaxis) BMT if not responding to therapy, sibling donor
37
What are the clinical features of CGD?
``` Respiratory infections Abscesses Hepatomegaly Suppurative lymphadenopathy S. aureus, serratia, aspergillus, nocardia, burkholderia ```
38
Which component of penicillin is responsible for a) most allergies b) anaphylaxis?
a) Major determinant | b) Minor determinant
39
Name 5 types of penicillin reactions
Delayed maculopapular exanthems Mucocutaneous syndromes such as Stevens-Johnson syndrome Drug fever Acute interstitial nephritis Pulmonary infiltrates with eosinophilia
40
Can patients with egg allergy safely receive influenza vaccine?
Yes
41
How do you confirm milk allergy?
Milk challenge
42
In most cases, does allergic rhinitis persist through to adulthood?
Yes
43
When do patients with allergic rhinitis typically develop symptoms?
Before age 20
44
What are causes of perennial rhinitis?
Molds Dust mite Animal dander Cockroach
45
What is the best treatment for perennial allergic rhinitis?
Most important: Avoid allergens - Seal mattress/pillow in encasing, - Wash bedding/stuffed animals weekly in hot water, - Removal of carpets, - Removal of pets (this is the only way to get rid of dander) - HEPA filters lower airborne mold. ``` Oral antihistamines (if runny) Nasal corticosteroids (if congested) Immunotherapy ```
46
What allergens cause non-IgE mediated anaphylaxis?
Cold
47
How long should patients with anaphylaxis be observed?
12 hours | 24 hours if severe respiratory distress, hypotension
48
List 4 preventative recommendations for anaphylaxis
- Educate about how to administer EpiPen, encourage to err on the side of caution - Keep EpiPen with child at all times (school, parent) with 2 doses available - Trigger avoidance!! - MedicAlert bracelets should be recommended - Referral to allergy re: testing, information, therapy
49
When does biphasic reaction occur in anaphylaxis?
Occurs in approximately 15 to 30% of cases within 4 to 8 hours
50
What is Radioallergosorbent Test | (RAST)?
In vitro test looking at specific IgE to specific allergens
51
What are the benefits of RAST over skin testing?
Do not need to discontinue antihistamines/immunosuppressants Can be used as an adjunct to skin testing to determine safety for oral challenge No risk of anaphylaxis Disadvantages: Low PPV Not as sensitive as SPT False positives associated with hyper IgE states
52
What are the benefits of skin testing over RAST?
More sensitive Higher PPV (less false positives) Preferred testing for food allergies
53
What are the indications for immunotherapy?
- Allergic rhinoconjunctivitis(seasonal or perennial) - Asthma triggered by allergen exposures - Insect venom sensitivity
54
What is the non-immunologic differential for recurrent infections?
- Cystic fibrosis - GERD with aspiration - Diabetes - Sickle cell - HIV - Nephrotic syndrome - Malignancy
55
Name 5 categories of primary immunodeficiencies
``` Humoural Combined Cell-mediated Complement Phagocytic ```
56
What are red flags for primary immunodeficiency?
1. ≥1 systemic bacterial infections (sepsis, meningitis) 2. ≥2 serious respiratory or documented bacterial infections within 1 year (cellulitis, abscesses, draining otitis media, pneumonia, lymphadenitis) 3. Serious infections occurring at unusual sites (liver, brain abscess) 4. Infections with unusual/opportunistic pathogens (Pneumocystis jiroveci, Aspergillus, Serratia marcescens, Nocardia, Burkholderia cepacia) 5. Infections with common childhood pathogens but of unusual severity 6. FTT 7. Chronic diarrhea 8. Persistent infections after receiving live vaccines 9. Chronic oral or cutaneous candida infections
57
Name 5 antibody defect immunodeficiencies
``` X-linked agammaglobulinemia CVID Transient hypogamaglobulinemia of infancy HyperIgM syndrome IgA deficiency ```
58
Name 2 cell-mediated immunodeficiencies
DiGeorge | CMC
59
Name 4 combined immunodeficiencies
SCID Wiskott Aldrich Ataxia Telangectasiae X-linked lymphoproliferative disease
60
Name 2 phagocytic defect immunodeficiencies
CGD | Leukocyte adhesion deficiency
61
How do you test for humoral immunity?
CBC, differential (WBC, lymphocyte count) ``` Quantitative tests: Quantitative immunoglobulins (IgG, IgA, IgM) Lymphocyte immunophenotyping (CD19, CD20) ``` Qualitative tests: Specific antibody titres (tetanus IgG (pre and post booster), measles IgG, rubella IgG, mumps IgG): tests protein antigen response Pneumococcal IgG (pre/post vaccine): tests polysaccharide antigen response Isohemagluttanin (Anti-A, Anti-B titres): tests IgM and polysaccharide response
62
How do you treat CVID?
IVIg replacement therapy 400-600mg/kg Q3-4 weeks Avoid live vaccines Do not usually give routine vaccinations, but this is debated
63
After what age can you assess polysaccharide responses?
2 years of age
64
What should you do if you need to give blood products to a patient with selective IgA deficiency?
Should measure IgA level and if <0.1 need IgA depleted blood products or triple washed blood products, medic alert bracelet
65
How would you work up cell-mediated immunity (T cell)?
CBC, differential ``` Quantity: Lymphocyte immunophenotyping (CD2, CD3, CD4, CD8) ``` ``` Function: Mitogen stimulation (incubate T cells with phytohemagluttinin and they should rapidly divide) Intradermal skin testing with heat-killed candida (older test, no longer used) ```
66
What is the immune defect in DiGeorge?
T cell numbers, mitogen response may be low Usually does not result in clinically significant immunodeficiency Usually grow out of it Delay live vaccines until T cell numbers/mitogen response improves
67
What should patients with T cell problems be advised?
No live vaccines CMV negative, irradiated blood products If exposed to chickenpox, may need VZIg Medicalert
68
How do you treat CMCC?
Supportive care | Antifungals
69
What is the immunologic defect in CMCC?
Impaired mitogen response to candida specifically | Normal immunophenotyping
70
Should you breastfeed a baby with SCID?
No Mom can pass maternal lymphocytes that baby could engraft (also CMV risk) Needs to be pumped and irradiated
71
What are the clinical features of Omenn's syndrome?
Erythroderma Lymphadenopathy HSM Abnormal T cell clones (many T cells, but they are proliferations of a few clones!)
72
How do you test for a phagocytic defect?
CBC, diff NOBI (Nitrozolium blue test is the old test) Measurement of adhesion markers (CD11, CD 15, CD 18-leukocyte adhesion deficiency)
73
How do you test for complement deficiency?
C3, C4 CH50, AH50 C1 esterase inhibitor (herediatry angioedema)
74
What is the immunologic abnormality in CGD?
Normal number of neutrophils, migration and adhesion But do not burst when stimulated Can’t kill catalase-positive bacteria (Staph, Serratia,Aspergillus, Cepacia, Nocardia, Salmonella, BCG)
75
What vaccines should patients with complement deficiencies receive?
Pneumococcal | Meningococcal
76
What is the cross-reactivity between penicillins and cephalosporins?
1-2%
77
What advice do you give for high risk infant (first degree relative with atopy) re: prevention of allergy?
Exclusive breastfeeding for at least 6 months Partially/extensively hydrolyzed formulas for those who cannot breastfeed No evidence of delayed introduction beyond 4-6 months (eggs, peanut, milk)
78
What is the most common food allergen in children?
Cow's milk
79
What percentage of children outgrow peanut allergies?
20%
80
What foods can you introduce to children with egg/milk allergy to decrease time to tolerability?
Baked egg and milk (70-80% of allergic children will tolerate it)
81
What are the 3 types of non-IgE mediated food protein allergy?
FPIES FFIAP FPE
82
What is the age of onset for FPIES?
1 day to 1 year
83
What is the age of onset for FFIAP?
Days to 6 months
84
What is the age of onset for FPE?
Up to 2 years
85
What are the food allergens implicated in FPIES?
CM, rice, soy, oat, egg (and many others)
86
What are the food allergens implicated in FFIAP?
CM, soy, wheat, egg
87
What are the food allergens implicated in FPE?
CM, soy, wheat, egg
88
What are the symptoms of FPIES?
Mostly vomiting, persistent diarrhea, bloody stool, shock, FTT
89
What are the symptoms of FFIAP?
Bloody stools, mild diarrhea
90
What are the symptoms of FPE?
Mostly diarrhea, some vomiting | Can look like post-infectious
91
What are the laboratory findings of FPIES?
``` Anemia Hypoalbuminemia Leukocytosis Thrombocytosis ?Methemoglobinemia ?Acidemia ```
92
What are the laboratory findings of FFIAP?
Rarely Hb, low albumin
93
What are the laboratory findings of FPE?
Anemia, hypoalbuminemia
94
What are the main differences between FPIES, FFIAP, and FPE?
FPIES is most severe and most likely transition to IgE allergy. Vomiting is prominent. Abnormal bloodwork. FPE is mild. Diarrhea is prominent symptom. FFIAP minimal blood in the stool, but appear well. Usually better by 12 months.
95
What do you use to treat dietary protein enterocolitis?
Extensively hydrolyzed formula (not SOY)
96
When do you rechallenge in dietary protein enterocolitis?
12 months
97
What is the natural history of FPIES, FFIAP, and FPE?
FPIES-resolve by age 3-5 FFIAP-resolves by 12 months FPE-resolves by 24-36 months
98
Do patients with FPIES transition to IgE mediated cow's milk allergy?
Yes, 35% do
99
What test can you do for allergic rhinitis to determine the allergen?
Skin test
100
What are the 3 WHO definitions of anaphylaxis?
1) Skin/mucosa involvement - Pruritus - Flushing - Angioedema - Hives ``` AND EITHER A) Respiratory -Dyspnea -Wheezing -Stridor -Hypoxemia ``` B) Hypotension/end-organ - Collapse - Syncope - Incontinence 2) 2 of respiratory, skin, GI, hypotension in a patient exposed to known allergen 3) Hypotension after known allergen
101
When can you do skin testing after anaphylaxis?
6 weeks after
102
What allergens cause direct mast cell activation?
Physical factors (cold, heat, exercise, sunlight) Ethanol Medications (opoids)
103
What are patients with selective IgA deficiency at risk for (List 2 things)?
- Increased risk of autoimmune diseases, malignancy, and malabsorption - Risk of anaphylaxis to blood products due to presence of IgA antibodies

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