Allergy/Immunology Flashcards
1
Q
Secondary Causes of PID
A
Meds (steroids, chemo, anti epileptics) Infections (HIV, TB etc), Structural (CF, indwelling catheter, impaired membrane integrity), Malignancy, Other (DM, renal insufficiency, protein losing enteropathy, malnutrition, asplenia)
2
Q
T cell Defect age of onset
A
2-6 months
3
Q
T cell typical bacteria
A
Gram + and -ve bacteria
Mycobacteria
4
Q
T cell typical fungi
A
Candida, PJP
5
Q
T cell defect sites of infection
A
sinopulmonary, GI, septicemia
6
Q
T cell typical viruses
A
CMV, EBV, parainfluenza, adenovirus
7
Q
Associated features of T cell defects
A
Omenn Syndrome, Disease post BCG or VZV vaccine
8
Q
B cell typical age of onset
A
> 6 months after maternal immunoglobulin is gone. Up until adulthood
9
Q
B cell typical bacteria
A
Encapsulated (streptococcus and haemophilus)
10
Q
B cell Typical Viruses
A
Enterovirus
11
Q
B cell Typical fungi/parasites
A
Giardia, cryptosporidium
12
Q
B cell sites of infection
A
otitis media, sinusitis, pneumonia, arthritis, meningoencephalitis
SINOPULMONARY
13
Q
B cell associated features
A
Autoimmunity, lymphoma
14
Q
Phagocytic age of onset
A
Early onset
15
Q
Phagocytic typical bacteria
A
staph, pseudomonas, serratia
16
Q
Phagocytic typical viruses
A
none really
17
Q
Phagocytic typical fungi/parasites
A
candida, aspergillus
18
Q
Phagocytic Sites of Infection
A
Deep abscesses (liver, lungs), gingivitis, osteomyelitis, skin
19
Q
Phagocytic Associated Features
A
Delayed cord separation, poor wound healing
20
Q
Complement age of onset
A
Any
21
Q
Complement typical bacteria
A
Pneumococcus and meningococcus
22
Q
Complement Sites of infection
A
Septicemia, meningitis
23
Q
Complement associated features
A
autoimmunity (can get a lupus like picture
24
Q
Humoral Assessment: Number
A
Quantitative Ig levels, albumin for secondary loss Lymphocyte subsets (evaluates B cell numbers, CD 19 cells)- flow cytometry
25
Humoral Assessment: Function
Specific antibodies to vaccines the patient was exposed to
| Isohemagglutinins (IgM antibody to blood group A and /or B)
26
Cellular Assessment: Number
Total Lymphocyte counts
| Lymphocyte subsets to evaluate T cell numbers (CD4 and CD8). Flow cytometry
27
Cellular Assessment: Function
lymphocyte proliferation in response to mitogens and antigens
TRECs
T cell receipt Beta repertoire
28
Phagocytic Assessment: Number
Neutrophil Counts
29
Phagocytic Assessment: Function
NBT or NOBI (for CGD)
| Measurement of adhesion markers: CD 11 and CD 18 (LAD)
30
Complement Assessment: Number
C1 esterase inhibitor (good for hereditary angioedema)
| Specific complement levels
31
Complement Assessment: Function
```
Total Hemolytic Complement (CH50)
Alternate Pathway (AH50)
C1 Esterase inhibitor function (for hereditary angioedema)
```
32
X Linked Agammaglobulinemia: pathophysiology
Mutation in Bruton Tyrosine Kinase
No B Lymphocytes in Blood: No lymph tissue, no immunoglobulin production
No lymph nodes or tonsils
33
XLA Age of onset
6-24 months (when maternal IgG disappears)
34
XLA Types of recurrent infections
otitis media, pneumonia, sinusitis, diarrhea, arthritis, meningitis, sepsis, skin infections
ENCAPSULATED BACTERIA (strep, haemophilus)
Enteroviral meningoencephalitis
35
XLA Diagnosis
Absent B cells (<2% of lymphocytes)
Absent IgG, IgA, IgM
Absent antibodies to vaccines
Genetic confirmation of BTK mutation
36
Management of XLA
Antibiotics for active infections
IVIg for life
Follow PFTs and CT: at risk for bronchiectasis
37
CVID Age of Onset
1st decade of life, 3rd decade of life
38
CVID Clinical features
Recurrent Infections, Autoimmunity, Malignacy
| Presence of lymph tissue *normal or enlarged*
39
CVID Infections
```
Recurrent sinopulmonary infections (encapsulated bacteria)- bronchiectasis and fibrosis in lungs
GI Infections (Giardia and campylobacter)
Atypical Bacteria (Mycoplasma, Ureaplasma joint infections)
```
40
CVID types of AI/Inflammatory processes occur in what % of patients
```
20-25% patients
Cytopenias
GI (IBD, pan gastritis, small bowel nodule lymphoid hyperplasia),
Arthritis
Granulomas (lung, liver, spleen, skin)
Thyroiditis
```
41
Is CVID at risk of malignancy?
```
Yes
Increased lymphoreticular and gastric malignancies
15% in aldutgood
Non Hodgkin's lymphoma
Gastric Carcinomas
```
42
CVID Lab
Decreased Serum IgG (normal or reduced IgA0
Normal or low numbers of B cells
Decreased/absent antibodies to vaccines
Low Isohemagglutinins
43
Management of CVID
Antibiotics to treat infections
IgG replacement for life ( DOES NOT DECREASE AI OR MALIGNANCY)
PFTs and Chest CTs
44
SCID Pathophysiology
Defective Tcell and B cell function
| Mutation in common gamma chain of IL-2 receptor on X chromosome (X LINKED)
45
SCID age of onset
2-6 months
| die in infancy without treatment
46
SCID Clinical Features
Persistent, recurrent, sever, opportunistic infections
Bacterial, fungal, viral, sinopulmonary, oral thrush, chronic diarrhea
FTT
Absent Lymph Nodes and Tonsils
Absent thymus
47
Diagnosis of SCID
Lymphopenia
Severely reduced T cell Numbers
B and NK cell numbers can be low, normal, or elevated
Low or absent T cell function (in vitro lymphocyte proliferation in response to mitogens and antigens(
Absent antibodies to vaccines
48
Management of SCID
IVIg
PJP Prophylaxis
Blood products should be CMV -ve and irradiated
No breast feeding if mother is CMV +ve
Avoid Live vaccines
Strict isolation
New immune system ASAP: HSCT. Gene therapy for some cases (ADA deficiency)
49
Wiskott Aldrich Syndrome Triad
Thrombocytopenia, Eczema, Recurrent Pyogenic Infections (encapsulated organisms)
Can also have autoimmunity
Increased risk of malignancy as well
50
Diagnosis of WAS
```
Thrombocytopenia (small too), lymphopenia
Variable IgG
High IgA
Low IgM
High IgE
Poor antibody responses to some vaccines
Decreased T cell function
```
51
Management of WAS
IVIg
PJP prophylaxis
HSCT
Monitor for AI and malignancy
52
Ataxia Telangiectasia mode of inheritance
AR
53
Ataxia Telangiectasia clinical features
Ataxia (starts as a toddler)
Progressive Neurodegeneration
Telangiectasia (face, conjunctiva, ear lobes)
Immune deficiency (only in 2/3, not as severe)
25% develop malignancy especially LYMPHOMA
Abnormal DNA repair...should avoid Xray and CT
54
Ataxia Telangiectasia type of malignancy
LYMPHOMA
55
Ataxia Telangiectasia Diagnostic Labs
```
Increased Alpha Fetoprotein
Decreased T cell numbers
Low or normal IgG
Absent IgA
Sometimes elevated IgM
Decreased T cell function
Can have poor antibody response to some vaccines
```
56
Ataxia Telangiectasia Management
```
Supportive (HSCT doesn't fix neuro problems)
Avoid Ionizing radiation
IVIg for those with humoral deficiencies
Genetic counselling (ATM increased risk of breast cancer)
```
57
DiGeorge Syndrome clinical features
```
CATCH-22
Cardiac Defects (interrupted aortic arch)
Abnormal Facial Features
Thymic Hypoplasia
Cleft Palate and midline abnormalities
Hypocalcemia
22q11 deletion
```
Also FTT, DD, Psychiatric issues
58
DiGeorge Syndrome Lab Features
Mildly reduced CD4 and CD8
Hypocalcemia
Normal or mildly reduced T cell function
May (rare) have poor response to vaccines
59
DiGeorge Syndrome Management
Usually only have mild T cell deficiency which improves with age.
Some have complete DiGeorge- severe T cell deficiency
Blood products should be CMV -ve and irradiated
Should not have live vaccines until immune competence is demonstrated
Screen parents too
60
Chronic Granulomatous Disease Genetics
Phagocytic Defect
Defect in NADPH oxidase
65% are X linked (others are AR)
61
CGD organisms
```
Susceptible to catalase positive pathogens
S. Aureus
Aspergillus
Nocardia
Serratia Marcesscens
Burkholderia Cepacia
Salmonella
```
62
CGD Clinical Features
Recurrent bacterial and fungal infections (suppurative adenines, pneumonia, abscess, osteo, sepsis)
Abscesses and granulomas (in organs)
Inflammation (IBD)
63
CGD Lab features
Normal or increased neutrophil numbers
| Abnormal NOBI or NBT
64
CGD Management
Antibiotics for infection
Antibacterial and Antifungal prophylaxis (septa and itraconazole)
Anti-inflammatory treatments if needed
HSCT
65
Hyper IgE Syndrome (Job Syndrome) Clinical features
AD mutation STAT3
Recurrent Abscesses in the skin, lungs (cold boils)
Mucocutneous Candidiasis
Eczema
Facial Features (deep set eyes, prominent forehead, broad nasal bridge, bulbous nose, coarse skin)
Delayed shedding of teeth
Bone fractures, scoliosis, joint hyperlaxity
66
Lab features HyperIGE
VERY high IgE >20 000
Eosinophilia
Normal IgG, IgA, IgM
Function: poor antibody response to vaccines
67
Management of Hyper IgE
Treat Infections
| Anti Staph prophylaxis (septra)
68
Leukocyte Adhesion Defects Pathophysiology
AR
| Deficiency in adhesion molecules (abnormal neutrophil migration and penetration into tissue)
69
LAD Clinical features
Delayed Separation of umbilical cord (>4 weeks)
Staph infections in 1st year of life (dental, gingivitis, intestinal)
NO PUS FORMATION
70
LAD Lab Findings
Neutrophilia
| Absent surface adhesion molecules CD11 and CD 18
71
LAD Management
Antibiotic treatment and prophylaxis
| HSCT
72
Complement Deficiencies Characteristics
Severe, recurrent or invasive infections with encapsulated bacteria
Defects in C1-C4: Rheumatic Diseases
Defects in C5-C9: Disseminated Neisseria Infections, meningitis
73
Management of Complement Deficiencies
Antibiotic Prophylaxis
| Immunizations (meningococcal and pneumococcal)
74
Lab Features of Complement Deficiencies
C3 C4 usually normal
Other complement levels more useful
Abnormal CH 50 (function)
AH 50 (alternate pathway function)
75
PJP prophylaxis is needed in what type of immune deficiency
Cell mediated (T cell)
76
Does Transient hypogammaglobulinemia need treatment?
No! Usually self resolves
77
Hyper IgM type of immunodeficiency
Combined Immunodeficiency
78
Hyper IgM Clinical features
Infections (bacterial in 1st year of life, opportunistic as well)
Autoimmunity
Liver Disease (sclerosis cholangitis, chronic hepatitis)
Malignancy (lymphoma)
79
Lab Features of Hyper IgM
```
High IgM
Low IgG
Low IgA
Normal T and B cells
50% have neutropenia
Poor antibody response to vaccines
```
80
Hyper IgM management
Immunoglobulin replacement therapy
PJP prophylaxis
HSCT (for immune deficiency and to prevent malignancy)
81
Cartilage Hair Hypoplasia
```
Short limbed dwarfism
combined immune deficiency
Short pudgy hands, hyper extensible joints, sparse hair
Hirschsprung disease
Immune: SCID to near normal
Increased frequency of malignancy
```
Management: immunoglobulin replacement to HSCT
82
IRAK 4 Deficiency
recurrent, sever, invasive infections
| Normal B and T, phagocyte and complement function
83
Chronic Mucocutaneous Candidiasis features
Persistent candida infection in the skin, nails and mucous membranes
Endocrine Autoimmunity
APCED
84
Hereditary Angioedema
Recurrent episodes of swelling to face, extremities and internal organs
No urticaria, pain, heat or itch
Deficiency in C1 esterase inhibitor (AD). Low C4 levels
85
Hereditary Angioedema Management
Concentrates of C1 inhibitor infusions during attacks
| Androgens for prophylaxis as well
86
ALPS
Autoimmune cytopenias
Lymphoproliferation
Increased malignancy
Management with immune suppression
87
Lab findings of ALPS
Anemia, Neutropenia, and/or thrombocytopenia
Elevated IgG
Elevated % of T cells not expressing CD4 or CD 8
Elevated B12 levels
Normal antibody responses to vaccines
Normal T cell function
88
IPEX
Immune Dysregulation
Polyendocrinopathy (IDDM)
Enteropathy
X linked
89
Epinephrine should be given via what route for anaphylaxis
IM
90
Dose of epinephrine for anaphylaxis
0.01mg/kg IM max dose 0.5mg
91
How often can you repeat epi for anaphylaxis
every 5-15 minutes
92
Why don't we give SC epi for anaphylaxis
Local vasoconstriction may inhibit absorption
93
When do we give IV epi for anaphylaxis
After 3 doses of IM epi
| Persistent hypotension despite fluid resuscitation
94
In patients who are on Beta Blockers and are not responding to epi in anaphylaxis...what can you give them?
Glucagon
Has inotropic and chronotropic effects...not mediated through beta receptors
IV bolus
95
Second line agents in anaphylaxis (5)
```
Salbutamol
Nebulized Epinephrine
H1 Antagonist (antihistamines)
H2 Antagonists (ranitidine)
Corticosteroids
```
96
When do most biphasic reactions occur?
Within first 4-6 hours
| 5-20% of patients with anphylaxis
97
What increases your risk of a biphasic reaction?
Delayed epinephrine
More than one dose of epi
Severe symptoms at presentation
98
Anaphylaxis teaching
Epi pen
Anaphylaxis Action Plan
Medical Identification Device
Consider 3 day course of antihistamines and corticosteroids
Avoidance of trigger if obvious from history
Referral to Allergist
99
Most common causes of anaphylaxis
Food, venom, medication
100
Skin prick testing advantages
Fast
More sensitive than serum specific IgE
High negative predictive value
Cost Effective
101
SPT disadvantages
False Positives
Affected by use of antihistamines and steroids
Risk of systemic reaction
Can not perform if skin site affected
102
Serum Specific IgE testing pros (RAST)
Not affected by antihistamines, steroids, montelukast
No risk of systemic reaction
Can be performed if skin disease
103
Serum Specific IgE testing cons (RAST)
False positives if elevated total IgE
Less sensitive compared to SPT
More expensive
104
Management of Food Allergy
```
Avoid trigger
OIT
Epi auto injector
Anaphylaxis Action Plan
Medical Identification Device
```
105
Who is at high risk of a food allergy?
Having a first degree relative with an allergic condition (asthma, eczema, allergies)
106
How to prevent food allergy in children
No dietary restrictions during pregnancy or breastfeeding
Exclusively breastfed for 6 months
Hydrolyzed formula if infant not breastfed
Don't delay introduction of allergenic foods
Regular ingestion of newly introduced foods
107
Who gets food protein proctitis
Exclusively breastfed infants ages 2-8 weeks
| Cow's milk protein, eggs, soy, corn
108
Clinical features of food protein proctitis
Blood tinged stools
| Otherwise healthy
109
Management and diagnosis of food protein proctitis
Skin testing and IgE not helpful
Eliminate food from moms diet or extensively hydrolyzed formula
By 9 months 95% will tolerate food
110
Food protein induced enterocolitis syndrome (FPIES) onset
1-4 weeks after introduction of food
111
FPIES trigger foods
```
CMPA
Soy
grains
rice
meat
poultry
egg
potato
legumes
```
112
FPIES S/Sx
Repetitive vomiting 1-3 hours after ingesting food
hypotension
relieved by 3 years usually
113
FPIES diagnosis
Skin testing and serum IgE not helpful
| Eliminate offending agent
114
Venom Allergy 3 types of reactions
```
Local reaction (erythema, swelling, pruritus. immediate onset, resolves in a day)
Large Local Reactions (localized erythema, swelling >15cm. Develops 12-48 hours after sting and resolves in 5-10 days)
Systemic Reaction (anaphylaxis, generalized cutaneous symptoms beyond the site of the sting)
```
115
Management of venom allergy
If local reaction: testing not indicated
Anaphylaxis: Epi pen, all require testing, Immunotherapy
Generalized cutaneous reactions (<16 no testing. >16 yo testing if high risk)
116
What is the cross reactivity between penicillins and cephalosporins?
2%
117
Urticaria/Angioedema onset
Minutes to Days after exposure
Resolves 1-2 days after stopping medication
Treat with antihistamines
118
Exanthemous Eruption onset
Days to 2 weeks after drug exposure
Diffuse fine macule and papule
Resolves 5-14 days after stopping medication
119
Fixed Drug Eruption features
```
Hyperpigmented plaques
Same site wth re exposure
Discontinue the medication
Topical steroids and antihistamines
Steroids if really severe
```
120
Erythema Multiforme Features
```
Target Lesions
Mucosal membrane involvement
Stop meds
Topical steroids, antihistamines
Steroids for severe cases
```
121
SJS/TEN features
1-3 weeks after drug exposure
Fever, sore throat
Blistering lesions and skin detachment (<10% SJS, >30% TEN)
Mucus membrane
Discontinue med and supportive treatment
Systemic corticosteroids and IVIG controversial
122
Drug rash with eosinophilia features
Onset 1-8 weeks after drug exposure
Rash, Eosinophilia, hepatic dysfunction, fever, facial angioedema, LAD
stop meds
steroids if bad
123
Serum Sickness
```
1-3 weeks after drug exposure
Rash, arthralgia, renal disease, fever
LOW COMPLEMENT
Stop med, NSAIDs, analgesia
Steroids or plasmapheresis for severe cases
```
124
Serum Sickness- LIKE
```
1-3 weeks after drug exposure
No renal disease
NORMAL COMPLEMENT
Stop med, NSAIDs, analgesia
Steroids for severe cases
```
125
Can you have flu vaccine if allergic to eggs?
Yes
126
Egg allergic patients...can they receive all vaccines?
MMR: receive as a single dose
Inactivated Influenza Vaccine: single dose normal monitoring
Live Attenuated Influenza Vaccine: single dose normal monitoring
Yellow fever: NEEDS SKIN TESTING BEFORE!
127
Chronic Urticaria Treatment
2nd gen antihistamine
second line: increase dose 4 fold
third line: add montelukast or cyclosporin or omalizumab
short course of steroids
128
Hereditary Angioedema Inheritance
AD
129
Hereditary Angioedema Pathphys
C1 esterase inhibitor deficiency/dysfunction
130
Allergic Rhinitis Treatment
```
Allergen Avoidance
Second gen antihistamine
Intranasal corticosteroids
LTRA
Immunotherapy
```
