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Immunology Flashcards

1
Q

X linked agammaglobulinema

A

Btk deficiency, pre B cell arrest

Presents 4-6mo, boys, 1:100,000
Absent B cells

Recurrent infections
-bacterial
-enteroviral
Absent lymphoid tissue

Ex IVIG

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2
Q

Drug reactions

A

Type 1- IgE mediated
Type 2- cytotoxic (haemolytic anemia secondary to ceohalosporin)
Type 3- immune complex ( serum sickness)
Type 4- delayed hypersensitivity ( cell mediated ; SJS/TEN ; rash)

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3
Q

Innate immune system

A

Cellular
- phagocytes (ROS by Resp burst and NAPDPH oxidase)

Neutrophils
eosinophils (IgE, helminth),
basophils (Mast cell, hypersens)
NK cells (IFN gamma) - cytotoxic to virus and tumor

  • macrophages
    Mediators between innate and adaptive
    Dendritic cells (APC) NB in recognition by T lymphocytes via MHC2

Humoral
- Complement

Rapid response 0-4hours; not antigen specific

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4
Q

Innate immune system defects

A

CGD
LAD
TLR defects
Complement deficiencies

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5
Q

Leukocyte adhesion defect

Impaired adhesion of leukocytes to vascular endothelium

A

Delayed cord separation
Often high WCC
Severe necrotising bacterial infections (staph and GNB) but NO PUS
Oral/genital infections

Absent expression of CD18 and CD11 on flow cytometry

HSCT

3 types
- LAD1 most common, defect Beta chain of beta2 integrin

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6
Q

Chronic granulomatous disease

A

Defect in phagocyte NADPH oxidase
Most common type is Xlinked (g91phox deficiency)
Inheritance XL OR AR
Increase incidence Maori boys

Clinical

  • recurrent severe infections with catalase positive organisms (mnemonic cats been places )
  • Burkhokderia ; -nocardia - pasturella. ; - aspergillus
  • Candida ; - E Coli - staph NB abscess; - serratia
Recurrent skin / GI infections
- pneumonic, abscess, Supp adenitis, ON, bacteraemia or fungaemia 
Abscesses (skin, liver, lung)
VEOIBD ; mimic IBD, form granulomas
Adenopathy
Hepatosplenomegaly

Inx
DHR or NBT

Rx 
Itraconazole fungal prophylaxis
PCP prophy (cotrimox)
IFN GAMMA
HSCT
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7
Q

Mononoclonal AB targets

A

Omalizumab - IgE
Infliximab - TNF alpha inhibitor (binds to it and inhibits it’s activity)
Rituximab - CD20 (b cells)
Alemtuzumab(Campath) - CD52(mono/lymphocytes)
Mepolizumab- IL5
Anankira - IL1R antagonist
Toculizimab - Anti IL6

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8
Q

HLH

PRIMARY
SECONDARY

A

Diagnosis
- molecular

- Clinical (5/8 of below)
Fever >38.5
Splenomegaly 
Cytopenia (2+cell line)
Hypertroglyceridemia +/_ low fibrinogen
Haemophagocytosis on BM
LOW NK activity
Ferritin >500
Elevated soluble CD25 ( IL2 receptor)
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9
Q

Adaptive immune system

A

B lymphocytes

T lymphocytes

Antigen specific
Amplification or response on re-exposure
Memory

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10
Q

CVID

A

1+

  • susceptibility to infection
  • autoimmune
  • granulomatous disease
  • unexplained polyclonal prolif
  • affected family member with Ab deficiency

AND
Marked decrease IgA, IgG +/- IgM
Poor vaccine response, low memory B cells

Diagnosis >4
Secondary causes ruled out
No evidence T cell deficiency

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11
Q

B cell disorders

Antibody production defects

A 
Present 4-6mo
Recurrent sinopulmonary infections 
- encapsulated bacteria
- parasites / fungi - giardia and cryptosporidium
- enterovirus (esp in XLA)

THI
XLA
CVID

HyperIgm( intrinsic T cell defect)
- CD40ligand ; risk PCP

Vaccine response

  • t dependent ( diphtheria, tetanus, PCV)
  • t independent (polysaccharide conjugate, prevnar23
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12
Q

X linked hyperIgM

A

Mutation CD40 ligand gene
Intrinsic T cell defect (CD40L on activated T cell binds CD40 on b cellls)

No isotope switching

Low IgG and igA, High IgM
Abnormal vaccine response

Recurrent infection
Risk PCP (40% wo Rx)
Cryptosporidium ( risk Sclerosing cholangitis)
Giardia

Rx
IVIG
PCP Prophy
Avoid live vaccines and crypto

BMT (70% survival )

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13
Q

Selective IgA deficiency

A

Common 1/500
Low IgA, normal IgG and IgM

Increase risk mucosal infections (Resp/GI)
Assoc atopy and autoimmune
Risk anaphylaxis with blood products

IVIG no use

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14
Q

T Helper subsets

A

Th1: IFN gamma ; macrophage activation and IgG production; role in autoimmune

Th2: IL4,5,13; mast cell, IgE, eosinophil production
Role allergy and parasitic infection

Th17; IL17,22 ; neutrophil, monocytic inflammation, role autoimmunity

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15
Q

Which cytokines cases T cell proliferation

A

IL-2
Role prolif of memory and effector T cells
Maintenance Treg

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16
Q

SCID

A 
Severe, fatal by 1yr
Combined (humoral and adaptive)
Immune def
- low lymphocytes ( <3 under 3 months) 
- T cell deficiency

Present <3 mo with recurrent infections, FTT, diarrhoea, thrush; opportunistic infection ( PCP)
No thymus, no lymph tissue

Inx
Newborn screening ( tests for TREC)
Lymphocyte subsets
Lymph proliferation testing (PHA)

Most common(45%) is common gamma chain (T-B+NK-) which is XLinked mutation JAK3)

Rx 
Isolation
Reduce CMV risk 
IVIG
PCP and fungal prophy
No live vaccines 
Caution transfusion ( cmv irradiated )

Rx
HSCT

17
Q

MHC

A

MhC1- cytotoxic T (CD8)

MHC2- Helper T(CD4)

18
Q

22q11 deletion ( DiGeorge)

A 
Conotruncal cardiac defect (interrupted arch, Truncus, TOF)
Thymic hypoplasia (lymphopenia)
- complete (absent) vs partial
Hypoparathyroid 
- low calcium
Palatal defects
Low IQ
Increase risk schizophrenia
Dysmorphic

Rx COmplete DGS

  • BMT( Hla Match)
  • thymus transplant
19
Q

Wiskott Aldrich

A

X linked mutation (WASP deficiency)

Immune deficiency
Eczema
Small platelets

Increase autoimmunity

20
Q

Ataxia telangiectasia

A

DNA repair defect

Immune defect
Neuro defect ( cerbellar ataxia, chorea)
Oculocutaneous telangiectasia

Low IgA
Higg AFP
ATM gene mutation

21
Q

Chediak Hegashi

A

Immune defect
Neuro defect
Oculocutaneous albinism

Giant lysosomal granules in neutrophils
Mutation LYS7 gene

Rx HSCT

22
Q

IPEX

A

Immune defect
Polyendocrinopathy
enteropathy
X linked

High eosinophils

23
Q

Defect IL-12 and IFN gamma

A

Increase risk mycobacterium infection

24
Q

HyperIgE

A 
Eczema
Recurrent skin infection
Cold abscesses ( staph)
Increase risk fungal infection ( low TH17)
Lung pneumatocoeles( AD)

AD - Job syndrome, STAT3 mutation

  • coarse facial features
  • pneumatocoeles
  • delayed shedding teeth
AR - Dock8 deficiency 
-autoimmune
-no coarse facial features 
- delayed shedding primary dentition
- marked viral skin infection (molluscum)
Elevated IgE
25
Q

Omenn syndrome

A

exacerbated response to oligoclonal T cells

Erythroderma
Diarrhoea, protein losing enteropathy

High IgE and eosinophils

Can occur in SCID with maternal engraftment causing GVHD like reaction

26
Q

Inflammatory cytokines

A

IL-1beta, IL6, TNF alpha

Cause rise in CRP
IL6- increase CRP
IL1 - fever

27
Q

Interleukins

IL2

IL12

IL17

A

L2(produce CD4 helper T) - increase CD4 and CD8 prolif

IL12 acts on NK cells and helps helper t to diff into TH1 ( increases INF gamma - antiviral)

IL-17 helps recruit neutrophils to site of inflamm

IL 4,5,10,13 related to allergy ; stimulate helper T to form TH2 - mast cell, eosinophil, basophils

IL10 and TGF Beta - Regulatory T cells

28
Q

Immunoglobulins

A

IgA - present in mucosal surfaces, secretions and breast milk. T1/2 6 days
Can activate alternative and lectin complement

IgG -80% serum, 4 subclasses, involved in opsonisation, activates classical complement pathway, binding NK. Cells for ADCC.
Only Ig that crosses placenta
T1/2- 7-23days

IgM- first antibody response, most effective in activating complement. T 1/2 5 days. Makes up 13% Ig

IgE - allergy and anti parasite

IgD- tells mature B cells when ready to leave BM

29
Q

Hereditary angioedema

A

Rare AD genetic disorder due to deficiency of C1 inhibitor. PRESENTS with unpredictable angioedema, refractory to adrenaline

3 types
Type 1 most common (85%, low C1INH)

Low C4
Family history
Recurrent angioedema without urticaria
Family history ( AD but 25% de novo mutations)

Triggers for attacks HAE

  • stress, anxiety, minor trauma
  • oestrogen, ACE-is

Present
recurrent severe abdo pain
Facial/lip swelling
No urticaria, not itchy

Rx
c1 inhibitor concentrate

30
Q

MHC 1

A

Bound by CD8 (cytotoxic T cells)
Codes HLA A,B,C
Found on all nucleated cells

31
Q

MHC

A

Bound by CD4 ( helper T cells)
Code HLA DR, DQ, DP
Only found on antigen presenting cells

32
Q

Chickenpox infectious period

A

2-3 days before spots appear until all spots crusted over

33
Q

CYP3A4 inducers

A 
Phenytoin, phenobarbitone
Carbamazepine
Rifampicin
St Johns Wort
Spironolactone
Smoker
Alcoholic
34
Q

CYP3A4 inhibitors

A 
Cimetidine
Ketoconazole, fluconazole
Erythromycin
Fluoxetine
Ciprofloxacin
Grapefruit juice
Amiodarone
Omeprazole
Indinavir
Mnemonic: Some Certain Silly Compounds Annoyingly Inhibit Enzymes, Grrrrrrr
Sodium valporate 
Ciprofloxacin
Sulphonamide 
Cimetidine/omeprazole 
Antifungals, amiodarone 
Isoniazid 
Erythromycin/clarithromycin 
Grapefruit juic
35
Q

In children with immunodeficiency, the purpose of routine irradiation of administered blood products is to decrease the risk of

A

GVHD

36
Q

Incontinentia pigmentations

A

XLD disease, seen mostly females (fatal males)

Null mutation in gene encoding NEMO

HYPOMORPHIC mutations in Nemo gene causes XL anhidrotic ectodermal dysplasia and immunodeficiency in males (Nemo defect - defect in NF-kB activation )

IP
- linear vesiculobullous lesions —> pigmented lesions along blaschko lines
Devel delay, seizures, ataxia
Hypodontia, abnormal eyes, nails, hair

Nemo gene sequencing

37
Q

TransfusionGVHD

A

Skin biopsy satellite dyskeratosis is pathognomonic

Panycytopenia
Liver dysfunction
GI

SKIN/bm/liver/gi

Paeds (25 decks)

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