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Immunology > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

Warning signs for adult primary immunodeficiency disorders

A

4 or more infections requiring anitbiotics within one year - otitis, bronchtiis, sinusitis, penumonia
Recurring infections or infection requiring prologned antibiotic therapy
2 or more severe bacterial infections - osteomyelitis, menignitis, speticaemia, cellulitos
2 or more radiologically proven pneumonia within 3 yrars
INfection w unusual localisation or pathogen
PID in family
FTT in paeds

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1
Q

Ass features primary immunodeficiency

A

Atypical eczema
Chronic diarrhoea
FTT
Telangiectasia - ataxic
Hepatosplenomegaly
Endocrinopathy
Chronic osteomyeltisi/deep seated abscess
Mouth ulcers
AI
FH

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2
Q

Scondary antibody deficiency causes

A

Etrems of age
Urameia
Toxins
Acute and chronic infections
Burns - lose skin
Myotonic dystrophy
Protein-losing states
Lymphangiectasia

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3
Q

Examination for immunodeficiencies

A

Weight and heigh t- FTT
Structural damage from infections w look or scan eg ears, lungs, sinuses
AI features - vitiligo, alopecia, goitre
Absent tonsils - XLA
lymphadenopathy
Hepatosplaenomegaly
Other potnetial diagnositc features - telagniectasia, eczema

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4
Q

Features of immunodeficiencies

A

Increased susceptibility to infection
Susceptibility to cancers
Increased AI diseases
What deficiency is

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5
Q

First investigations for immunodeficiencies

A

FBC - individual white cells not just overall count
IgG, IgA, IgM
Serum electrophoresis
Lymphocyte surface markers - T cell (CD4/CD8), B cells, NK cells, B cell subclasses - naive, memory, class switched
Specific antibody responses

Later - genetics, complement AP100/CH100, neutrophil burst assay, lymphocyte prolif assay

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6
Q

Complement investigation

A

AP100/CH100

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7
Q

Neutrophil investigation

A

Neutrophil burst assays - can go oxidative burst to kill pathogens?

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8
Q

Lymphocyte investigations

A

Lymphocyte proliferation assays - if can proliferate on exposure to pathogen

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9
Q

Humoral deficiency presentations

A

Antibody deficiency
Recurrent sinopulmonary infections
Chronic GI infections
Bactaraemias
Meningitis
Como pathogens incl encapsulated bacteria

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10
Q

Common pathogens causing humoral deficiency

A

S.pnuemoniae, H.influenzae type B, N.meningitidis
Giardia, cryptosporidia, campylobacter

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11
Q

Conditions causing humoral (antibody) deficiency

A

Selective IgA def
Common variable ID
Specific AB D
XLA
X lymphoprolif disease
Hyper IgM syndrome
Hyper IgE syndroe

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12
Q

What immunodef is most common

A

Hypogammaglobulunaemia

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13
Q

Size of Igs

A

IgM - pentameter
IgA - dimer
IgG - monomer

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14
Q

What Ig deficient if losing through gut or kidney

A

IgG - monomer - smallest

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15
Q

When is low IgM noraml

A

Older age/normal variation
V low in lymphoprolif disease

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16
Q

How assess functioning of Igs

A

Response to vaccines
baseline serology
give pneumovax vaccine
assess antibody level weeks later
Use pneumococcal IgG antibodies

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17
Q

Which vaccines are easier to make an immune repsonse to

A

Proetin
Prevenar can be used if necessary for antibody respone testing but elss helpul than pneumova

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18
Q

How diagnose sepcific antibody deficiency

A

Pneomovax vaccination - below 4 x greater antibodyes 4-6 weeks later than baseline
Check at 6 months in case of loss

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19
Q

What measure if use conjugated pneumococcal vaccine to test for specific antibody deficinecy

A

Number of serotypes immune system responded to -
normal = >7 >0.35mcg/ml

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20
Q

Causes of reduced production of immunoglobulins

A

Primary ID - CVID, XLA - genetic
Medications
Malignancy - multiple myeloma, lymphoma, leukaemia
Systemic illness - sev infection -> bone marrow supression

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21
Q

Medications causing reduced production of immunoglobulins

A

Prednisolone
Azathioprine
Methotrexate
Rituximab
Anti-epileptics
Antipsychotics

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22
Q

Causes o increased loss of Igs

A

Nephrotic syndrome
Coeliac disease,
IBD
Peritonneal dialysis

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23
Q

How diagnoise CVID

A

> 4 years old
Low IgG
Low IgA+/- IgM
Poor or absent repsonse to immunisation
Exclusion of other immunodeficiencies

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24
Q

Treating low IgG levels

A

Reverse underlying cause
Give prophylactic antibiotics
Vaccination - avoid live vaccines!
Replace immunoglobulin - IV or subcut, ONLY IgG GIVEN

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25
Q

What immunoglobulin can be replaced by IV/SC

A

IgG

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26
Q

Dosage and goal of IgG replacement

A

0.4g/kg/month
Target trough level IgG>6g/L - shoudnt go below

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27
Q

Prophylacitv antibiotics in CVID

A

Azithromycin
Co-trimoxazole

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28
Q

How do cell mediated immunodeficiencies present

A

Infections with ordinarily ‘benigng’ viruses, opportunistic IC pathogens, fungi

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29
Q

Common pathogens causing infection in cell mediated immunodeficiency

A

CMV, EBV, herpes virus
Mycobacteria
Fungo - candida, cryptococcus, oneumocystisis

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30
Q

Diseases casuing cell mediated immunodeficiency

A

SCID
Chronic mucutaneous candidiasis
Di george syndrome
Wiskott aldrich syndrome

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31
Q

What cells are affected in SCID

A

T cells always +/- B cells +/- NK cells

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32
Q

Genetic causes of chronic mucocutaneous candidiasis

A

AIRE deficiency, Dectin-1, CARD-9

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33
Q

What causes di george syndrome

A

22q11.2 deletion -> absent thymus -> T cell lymphopaenia

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34
Q

Deficiencies of innate immunity

A

Phagocyte disorders
NK cell defects
Cytokine disorders
Toll-like receptors
Complement deficiency

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35
Q

Complement functions

A

Ability to lyse cells
Ability to opsonize particles (easier to engulf)
When activated -> inflammatory proteins

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36
Q

What complement pathway is antidbody indpendent vs depndent

A

Classical = antibody triggeres
Alternate = pathogen triggers - antibody independent

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37
Q

Complemetn oathways outcome

A

C3 -> membrane attack complex -> transmembrane pore -> cell lysis

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38
Q

Classical complement pathway deficiency what causes

A

C1,C2,C4 def -> systemic AI disease incl SLE

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39
Q

What bacteria does classical complement deficiency increase risk infection of

A

Encapsulated bacteria eg S.pneumonia, H.influenzae, Neissieria meningitidis

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40
Q

What causes alternate pathway deficneicny

A

Properdin deficiency - regulates C3 and C5 convertases - if deficient rapud decay of convertases, less efficeient, cant do pathway

41
Q

Features of alternate pathway deficinecy

A

X linked
Recurrent severe bacterial infection in childhood eg meningitis due to neissieria meningitisides - ifmore than one = red flag

42
Q

What causes lectin oathway deficiency

A

Mannan binding lectin

43
Q

Features of lectin pathway deficiency

A

Increased risk encapsulated bacteria < 2 years
Asymptomatuc in adults - adaptive immune system overtakes
In CF - worse prognosis

44
Q

Presentation of C3 deficiency - common pathwya deficiency

A

Life threatening recurrent infections starting in early childhood
Particuarly susceptible to encapsulated organsisnsm
Ass w immune complex disease - 1/4 - renal disease, AI disease eg SLE

45
Q

MA complex deficiencies what is it

A

Deficiency of any terminal componenets complement -> susceptibility to gram negative bateria esp neisseria species

46
Q

What does CD59 deficiency cause pathology

A

CD59 is inhibitory protein for MAC complex to our own cells
If deficient -> lysis of cells

47
Q

What condition is caused by CD59 deficiency due to aquired PIGA gene mutation

A

Paroxysmal nocturnal haematuria

48
Q

PNH presentation

A

Red urine first thing in morning- Haemolysis as urine condensed overnight red most obvious in morning
Anemia - tired, breathless
Increased thrombosis incidence
Rare and life threatening

49
Q

What does Radial immunodifusion assays CH200/AP100 do

A

Classical pathway complement - CH100
Alternative pathway complemetn - AP100

50
Q

Results of CH100/AP100 normal

A

Clear zone formed around well - patient serum has lysed foreign RBC in agar

51
Q

What results indicate alternate vs classical pathway deficiency AP100/CH100

A

Lysis in CH100 assay w no lysis AP100 = alternate deficiency
Lysis in AP100 w no lyssi CH100 = classical deficiency

52
Q

What results in AP100/CH100 suggest terminal componenet deficiency

A

No lysis in AP100 or CH100

53
Q

Investigations for complement deficiency

A

AP100/CH100
If abnormal total lysis assay -> C3/C4 complement measuring
Genetics

54
Q

How does neutrophil/grnaulocyte deficiency present

A

Recurrent invasive skin and soft tissue infetions
Focal abscesses requiring incision and drainage esp in llvier/lung
Granulomas/granulomatous disease

55
Q

Common pathogens in neutrophil deficiency

A

S.aureus, gram negative bacilli
Aspergillus, nocardia

56
Q

Priamry immunodeficiencies causing granulocyte/neutropgil deficiency

A

Chronic granulomatous disease
Leukocyte adhesion afects
Chediak Higashi dyndrome

57
Q

Key features of chronic granulomatous disease

A

Early presentation
Skin abscess and deep seated lung, lymph, liver and bone infections
Linked to IBD

58
Q

pathogens seen in chronic granulomatous disease

A

Catalase + bacteria - staph, kelbsiella, serratia and burkholderia
Fungal - aspergillus

59
Q

Neutrophil oxidative burst assay

A

Stimulate neutrophils to oxidative burst - if cant do so problem
Healthy control - peak will shift on graph
Patient - peak stays the same

60
Q

Carrier of neurtophil deficiency

A

50% of cells can undergo oxidative burst, 50% cant
X linked or autosomal recessive, can pass on

61
Q

Treatment for CGD

A

Prophylactic antibiotics and antifungals
Interferon gamma
Bone marrow traansplantion
?Gene therapy?

62
Q

Bcterial pathogens seen in combined T and B cell deficiencies

A

Intracellular mycobacteria
Salmonella spp

63
Q

Fungal in combined T and B cell deficiencies

A

Candida spp
Aspergillus spp
Cryptococcus neoformans

64
Q

Protozoal

A

Pneumocystitis carinii
Toxoplasma gondii
Cryptosporisia

65
Q

Viral causes of infection in combines T and B cell deficiencies

A

RSV, parainfluenzae
GI - rotavirus, norovirus
CMV, adenovirus

66
Q

What would cryptosporidium infection suggest about immunodeficiency cause

A

CD40 ligand deficinecy =hyper IgM syndrome and liver dysfunction

67
Q

CGD disease what infection think about

A

Fungal disease in pneumonia or pneumonitis
Deep seated abscess eg in liver - S.aureus

68
Q

Pathogen idenitificanion in immunodeficiency

A

Disease
Srology - materangl Ig, Ab deficiency
PCR
Timeline
Which site - imaging
Tissue biopsy
Which strain/serotype - vaccine strain
Is it a contaminant
Dual oathology - more than one

69
Q

Treatment for fungal infection in extremely neutrophil deficient patient

A

Leukocyte orneutrophil infusions acutely

70
Q

What prevent or treatment for RSV in SCID

A

Palivuzumba - anti RSV monoclonal antibody

71
Q

Immunosupressive drugs

A

Steroids
Ciclosporin
Sirolimus
Monoclonals - anakinra, infliximab

72
Q

Curative therapy for immunodeficiency

A

Haematopoietic stem cell transplant
Gene therapy - addition, editing, stem cells, target cells - uses viral vectors
Thymic transplant
Enzyme replacement therapy - adenosine diamentes or DASKD - not long term but to prep for trnasplant

73
Q

How bone marrow donor taken

A

GSF harvesting stem cells -> mobilise stem cells into peripheral bood and mkae easier
Otherwsie from bone marrow

74
Q

Why need chemo if bone arrow in receiver patient transplant

A

Make space for donor cells in their bone marrow

75
Q

SCID prognosis

A

<12 months if no ucrative therapy

76
Q

How does bone marrow transplantation improve survival

A

<5 years = same as avg population
>5 years still v good survival rate
even prophylactic antibiotics only 50% survival by late 20s early 30s

77
Q

What matching need for bone marrow transplant

A

Sibling ideal
Half identical/hablo - parent

78
Q

Risk in using habloidentical n=bone marrow transplant and how prevent

A

graft vs host disease
T cell depletion

79
Q

T cell depletion how done

A

CD3/TCRalpha-beta/CD19 depletion- TCR gmma T cells left- antileukaemia
OR replete marrow w post infusion cyclophosphamide

80
Q

Can you use a non matched donor of bone in transplant

A

Yes if do T cell depletion

81
Q

Gene addition therapy how works

A

Bone marrow cells removed and parent cells of immune system isolated
Cells infected w virus w corrected gene -> intracellular, faulty gene replaced with corrective viral gene
Cells returned -> 2 weeks later immune system protected

82
Q

Problem with gene addition therapy

A

Can stimulate an oncogene -> clonal proliferation of cell -> leaukaemia or myelodysplasia
Now use safer retroviral vectors w self inactivation built in

83
Q

What conditions is thymus transplantation used in

A

Di george
Charge syndroe

84
Q

Why is early diagnosis of primary ID important

A

older patients -> organ damage from mulitple infections

85
Q

Major comorbidities form XLA

A

Lung diseases and bronchiectasis

86
Q

FEATURES OF PRIMARY DEFICIENCY

A

lifelong history of infections
Younger age of onset
Concurrent AI or malignancy
FH

87
Q

FEATURES OF SECONDARY ID

A

Use of chemo or immunosupressants
Radiation
Malignancy
Protein loss from gut or kindeys
Malnourishment
HIV

88
Q

Bloods in immunodeficiency

A

FBC
U+Es
LFTs
B12
Thyrod function
HBA1c
Serum immunoglobulins and electrophoress
HIV
lymphocyte subsets
Sepcific viral antibodies and bactreria eg varicella, MMR, tetanus, pneumococcus

89
Q

Imaging for structural damage immunod

A

HRCT - bronchiectasis, thymoma, malignancy

90
Q

What test need to do before give azithromycin

A

ECG - QT interval
2 sputum cultures to rule out acid fast bacilli which could be masked by azithromycin

91
Q

Tests done before give IVIG

A

Screening for HIV, hep B and hep C - cant test after as affected
Also test isohaemmaglutinins IgM against blood group antigens

92
Q

Basic panel in flow cytometry

A

Number of total lymphocytes
T cells
B cells
NK cells
CD4 and CD8 T cells

93
Q

What is cellular toxicity of NK cells and cytotoxic T cells a feature of

A

Haemophagocytic lymhohistiocytosis - uncontrolled proliferation of activated lymphocytes and histiocytes

94
Q

Which diseases have high polyclonal IgM levels

A

Liver diseases and other inflammatory conditions

95
Q

What causes raised IgA

A

Elderly
Chronic infection
Inflammation

96
Q

What can cause high polyclonal IgG

A

Reactive state
Sjrogens syndrome
HIV

97
Q

Why need electrophoresis in Igs interpretation

A

Ig level cant tell whether its monoclonal or polyclonal increase - electrophoresus can detect paraproteins and differentiate

98
Q

> 50,000 IgE what is it

A

Hyper IgE syndrome

99
Q

What is immunofixation

A

Stained paraprotiens for heavy or light chain and run electrophoresis

100
Q

Conditions causing overactive complemetn

A

C3G glomerulonephropathy
Atypical haemolytic uraemic syndrome

Immunology (4 decks)

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