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FRACP > Immunology > Flashcards

Immunology Flashcards

1
Q

Cd4 and 8 cells are otherwise known as?

A

Cd4 T helper

Cd8 cytotoxic T

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2
Q

What CD number is given to B cells and NK cells

A

B-19 and 20

NK-56

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3
Q

How many resp infections are seen in a normal child in a year
What might increase it

A

8-12

Daycare/school age self or sibling

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4
Q

What is the most common subgroup of immunodeficiency
What type of organisms usually infect
When do they usually present? Why?

A

B cell defects

Encapsulated organisms- pneumococcus HIB
Giardia
Enterovirus

After 6 months- maternal IgG has run out

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5
Q

How are B cell deficiencies treated

What is the half life

A

IVIG

30 days

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6
Q

What happens lymphoid tissue in B cell defects

A

Absent unless CVID

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7
Q

What are associations with B cell immunodeficiency

A

Reduced growth

Increased allergy and autoimmune disease

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8
Q

What is selective igA deficiency
How does it present
How frequently is it seen

A

Normal b and t cells but reduced igA
80% are asymptomatic, others like B cell defects
1:500 people

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9
Q

Does selective igA deficiency need IVIG

A

No!

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10
Q

Why should selected iGA deficiency have washed blood products
Which autoimmune disease is highly likely

A

High risk of transfusion reactions

Coeliac disease

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11
Q

What is the significance of IgG deficiency

A

Unknown!

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12
Q

What is x linked agammaglobulinaemia otherwise known as
What happens physiologically
What is seen on bloods

A

Brutons disease
Lack bruntons tyrosine kinase
B cells can’t mature from bone marrow
Low iGA, M and G and low circulating mature B cells with poor vaccine responses

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13
Q

What is x linked agammaglobulinaemia linked to

A

Inflammatory bowel disease

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14
Q

Hyper iGM syndrome
What is the pathology
What is the usual inheritance

A

Can’t switch IG subclasses- lack CD40 ligand
Lots of iGMs but lack the rest

X linked recessive

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15
Q

Hyper igM syndrome
What other infections do they get
What other abnormality do you see on blood tests
How therefore may they present

A

PCP
Neutropenia
Suspicion of HIV

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16
Q

Which B cell defect can have a fatal response to mono

A

Duncan’s- x linked lymphoproloferatove disease

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17
Q

What is hyper igE syndrome otherwise know as

What are 2 common features other than immune deficiency

A

Jobs syndrome
Severe eczema
Double rows of teeth

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18
Q

When does transient hypogammaglobulinaemia of infancy usually resolve by
Do they have normal B cell numbers and vaccine responses

A

2 yrs

Yes!

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19
Q

What immune cells are affected in CVID
When do they normally present?
What are they at risk of
What is seen on bloods

A

B can’t transform into T
Adolescence/ adults
Lymphomas and autoimmune diseases

Normal b and T cell numbers
Low IgG, M and A
May be pancytopenic

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20
Q

CVID
What happens to their lymphoid tissues
Is it more common in boys?

A

Increases- tonsils and spleen

No!

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21
Q

CVID
What defines severity
Do they get granulomas

A

Severity of the T cell defect

Yes- non caseating

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22
Q

What 5 tests are useful in looking at T cell defects

A 
Lymphocyte count 
Cxr? Thymus 
Lymphocyte subsets (B/T/NK)
Lymphocyte phenotypes (CD4,8,19,20)
Lymphocyte function to stimulation
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23
Q

T cell defects

What types of organisms do they get

A

Opportunistic
PCP
CMV
Candida

24
Q

What percentage of velocardiofacial syndrome have complete thymic hypoplasia

A

25%

25
Q

SCID
What test is used in newborn screening
What is seen most profoundly on bloods

A

T cell receptor excision circles

Lymphopenia

26
Q

SCID
Will they have normal lymphoid tissue
What is seen on cxr

A

No- reduces or absent

Absent thymus

27
Q

SCID
Which gene defect has available treatment
What is the most common genetic mode of inheritance and gene involved

A

ADA

X linked-IL2

28
Q

WAS
What is the gene involved and inheritance
What is the usual triad
What malignancy is more likely

A

WASP x linked recessive
Thrombocytopenia eczema and immunodeficiency
Lymphomas

29
Q

WAS

What is the pattern of immuglobulin changes

A

High igE and A
Low igM

(High ethanol alcohol, low mental state )

30
Q

SCID

how do they present if not found on screening

A

Severe diarrhoea and FTT
Opportunistic infections
GVHD

31
Q

What stain is positive on lung biopsy in PCP

A

Methenamine silver nitrate

32
Q

What rash is seen on omen syndrome

Which gene is involved

A

Erythroderma with skin peeling

RAG gene

33
Q

Which ataxia syndrome is associated with b and T cell issues

A

Ataxia teliengiectasia

34
Q

What type of infections are seen in phagocytic disease

Which organisms are seen

A

Gingivitis and ulcers
Abscesses
Deep and soft tissue infections.
Staph pneumococcus and aspergillus

35
Q

What are the 2 best tests for phagocytic diseases

A

Neutrophil count

Dihydroamine flow assay (neut function test)

36
Q

What is the usual pattern of inheritance for chronic granulomatous disease
What does it mimic

A

X linked recessive

Crohn’s disease

37
Q

Chronic granulomatous disease

What is seen in wcc and igg

A

Raised WCC and IgG

38
Q

Chronic granulomatous disease

What enzyme is lacking

A

Lack in NADPH Oxidase

39
Q

What is wrong with the lymphocytes in LAD 1 and 2
What is seen with the WCC
What is likely to be seen after birth

A

1- can’t adhere
2- cant roll
Always elevated
Delayed cord separation

40
Q

Which disorder of phagocytes presents with metaphyseal dysplasia

A

Cartilage hair dysplasia

42
Q

Chediak higashi
Phenotypically what is seen
With us the issue with the white cells
What other 2 things do they get

A

Red hair or albinism
Wcc granules are too big, can’t degranulate
Leukaemias and peripheral neuropathy

43
Q

Which gene is common in cyclic neutropenia

How frequently should bloods be done

A

ELANE

Twice weekly neuts for 6 weeks

44
Q

What is the pathological cause of hereditary angiodema

A

C1 esterase deficiency

45
Q

What complement is involved in sle

What infections are seen

A

C3

Encapsulated

46
Q

In late complement pathway defects what infections are commonly seen
What other rare defects can also give this

A

Recurrent neissera

Properidin def, factor D def

47
Q

Which test assesses classical and terminal complement pathways
Alternative

A

CH50

Ah50

48
Q

What are interleukins?

A

Types of cytokines- pro inflammatory

49
Q

What do the following activate

1,2,3,4,5

A 
1- macrophages- fever 
2-T - T cells 
3-b- B cell activation 
4- igE- class switching 
5- eosinophils iGA
6- CRP

Hot T BonE stEAk

50
Q

Antibodies
What are they made up of
What is the antigen biding portion called
What are the 2 regions?

What are the half lives for the following
IgA,M,g,E
Which crosses breast milk
Which crosses the placenta

A

2 heavy and 2 light chains
FAB region
Constant and variable portions

IGA-7 days
IgM- 5 days
IgG 21 days
IgE 3 days

IgA
IgG

51
Q

Which 2 immune cells do you need for class switching

A

CD40 and helper T cells

52
Q

What is the half life of IVIG

A

30 days

53
Q

Which MAB is a terminal pathway inhibitor

A

Ecilizumab

54
Q

Which antibody activates the classical complement pathway? alternative?

A

Classical- ab/ag complexes

Alternative- igA

55
Q

What is the best test for hereditary angiodema?
During an attack what 3 things go low
How is it treated

A

C4 level and c1 inhibitor activity
C2,4 and CH50
C1 inhibitor concentrate

56
Q

What pathway is involved In Mendelian susceptibility to mycobacterium disease

A

Il12

FRACP (23 decks)

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