Immunology Flashcards by Rachel Longwell

Cd4 and 8 cells are otherwise known as?

Cd4 T helper

Cd8 cytotoxic T

How well did you know this?

Not at all

Perfectly

What CD number is given to B cells and NK cells

B-19 and 20

NK-56

How well did you know this?

Not at all

Perfectly

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

8-12

Daycare/school age self or sibling

How well did you know this?

Not at all

Perfectly

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

B cell defects

Encapsulated organisms- pneumococcus HIB
Giardia
Enterovirus

After 6 months- maternal IgG has run out

How well did you know this?

Not at all

Perfectly

How are B cell deficiencies treated

What is the half life

IVIG

30 days

How well did you know this?

Not at all

Perfectly

What happens lymphoid tissue in B cell defects

Absent unless CVID

How well did you know this?

Not at all

Perfectly

What are associations with B cell immunodeficiency

Reduced growth

Increased allergy and autoimmune disease

How well did you know this?

Not at all

Perfectly

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

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

How well did you know this?

Not at all

Perfectly

Does selective igA deficiency need IVIG

No!

How well did you know this?

Not at all

Perfectly

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

High risk of transfusion reactions

Coeliac disease

How well did you know this?

Not at all

Perfectly

What is the significance of IgG deficiency

Unknown!

How well did you know this?

Not at all

Perfectly

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

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

How well did you know this?

Not at all

Perfectly

What is x linked agammaglobulinaemia linked to

Inflammatory bowel disease

How well did you know this?

Not at all

Perfectly

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

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

X linked recessive

How well did you know this?

Not at all

Perfectly

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

PCP
Neutropenia
Suspicion of HIV

How well did you know this?

Not at all

Perfectly

Which B cell defect can have a fatal response to mono

Duncan’s- x linked lymphoproloferatove disease

How well did you know this?

Not at all

Perfectly

What is hyper igE syndrome otherwise know as

What are 2 common features other than immune deficiency

Jobs syndrome
Severe eczema
Double rows of teeth

How well did you know this?

Not at all

Perfectly

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

2 yrs

Yes!

How well did you know this?

Not at all

Perfectly

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

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

How well did you know this?

Not at all

Perfectly

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

Increases- tonsils and spleen

No!

How well did you know this?

Not at all

Perfectly

CVID
What defines severity
Do they get granulomas

Severity of the T cell defect

Yes- non caseating

How well did you know this?

Not at all

Perfectly

What 5 tests are useful in looking at T cell defects

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

How well did you know this?

Not at all

Perfectly

T cell defects

What types of organisms do they get

Opportunistic
PCP
CMV
Candida

What percentage of velocardiofacial syndrome have complete thymic hypoplasia

25%

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

T cell receptor excision circles | Lymphopenia

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

No- reduces or absent | Absent thymus

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

ADA | X linked-IL2

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

WASP x linked recessive Thrombocytopenia eczema and immunodeficiency Lymphomas

WAS | What is the pattern of immuglobulin changes

High igE and A Low igM (High ethanol alcohol, low mental state )

SCID | how do they present if not found on screening

Severe diarrhoea and FTT Opportunistic infections GVHD

What stain is positive on lung biopsy in PCP

Methenamine silver nitrate

What rash is seen on omen syndrome | Which gene is involved

Erythroderma with skin peeling | RAG gene

Which ataxia syndrome is associated with b and T cell issues

Ataxia teliengiectasia

What type of infections are seen in phagocytic disease | Which organisms are seen

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

What are the 2 best tests for phagocytic diseases

Neutrophil count | Dihydroamine flow assay (neut function test)

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

X linked recessive | Crohn’s disease

Chronic granulomatous disease | What is seen in wcc and igg

Raised WCC and IgG

Chronic granulomatous disease | What enzyme is lacking

Lack in NADPH Oxidase

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

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

Which disorder of phagocytes presents with metaphyseal dysplasia

Cartilage hair dysplasia

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

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

Which gene is common in cyclic neutropenia | How frequently should bloods be done

ELANE | Twice weekly neuts for 6 weeks

What is the pathological cause of hereditary angiodema

C1 esterase deficiency

What complement is involved in sle | What infections are seen

C3 | Encapsulated

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

Recurrent neissera | Properidin def, factor D def

Which test assesses classical and terminal complement pathways Alternative

CH50 | Ah50

What are interleukins?

Types of cytokines- pro inflammatory

What do the following activate | 1,2,3,4,5

``` 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

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

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

Which 2 immune cells do you need for class switching

CD40 and helper T cells

What is the half life of IVIG

30 days

Which MAB is a terminal pathway inhibitor

Ecilizumab

Which antibody activates the classical complement pathway? alternative?

Classical- ab/ag complexes | Alternative- igA

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

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

What pathway is involved In Mendelian susceptibility to mycobacterium disease

Il12