Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Year 5 Pathology > Immuno > Flashcards

Immuno Flashcards

1
Q

Name three constitutive barriers to infection

A

Skin

  • packed keratinised cells
  • low pH, low oxygen tension
  • sebaceous glands; oil repels water and organisms

Mucosal surfaces

  • mucous physical barrier with lysozymes
  • cilia traps pathogens and helps remove

Commensal bacteria

  • compete with naughty organisms
  • produce fatty acids inhibit other pathogens
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Which cells assist in the innate immune system and how?

A

Polymorphonuclear cells/granulocytes

  • neutrophils, eosinophils, basophils
  • release enzymes, histamine, detect immune complexes

Monocytes and macrophages

  • circular in blood and migrate to tissues to differentiate to macrophages
  • present antigens to T cells

Natural killer cells
- cause lysis of naught cells, cytotoxic; kill ‘altered self’

Dendritic cells
- peripheral tissue, detect immune complexes, express cytokines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

By which mechanisms does the innate immune system work?

A

Phagocyte recruitment
- macrophages, neutrophils, dendritic cells all attracted to chemokines released in inflammatory process

Recognising microorganism
- toll-like receptors and mannose receptors to help body trigger immune complexes

Endocytosis
- OPSONIN bridges pathogen and phagocyte receptor to allow for eating

Phagolysosome
- pathogen killed in phagosome which fuses with lysosome, cheeky

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Oxidative vs non-oxidative killing

A

Oxidative

  • NADPH oxidase converts oxygen to ROS
  • Myeloperoxidase catalyses production of hydrocholorous acid, effective oxidant and anti-microbial

Non-oxidative

  • relase of lysozyme, lactoferrin in phagolysosome
  • enzymes in granules
  • broad coverage of bacteria and fungi
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Main components of the adaptive immune system

A

Humoral immunity
- B cells and antibodies

Cellular immunity
- T cells (CD4 + CD8)

Soluble components
- cytokines and chemokines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Which T cells recognise which HLA class?

A

CD4+ T cells = HLA II (4x2 = 8)

CD8+ T cells = HLA I (8x1 = 8)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Compare CD4+ and CD8+ T cells

A

CD4+

  • recognise peptides HLA II
  • trigger expression of cytokines
  • develop full B cell response

CD8+

  • recognise peptides HLA I
  • kill cells directly via perforin and granzymes, Fas ligand
  • secretes cytokines
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How are B cells activated?

A

B cell receptor Ig binds to antigen

Some mature to plasma cells secreting IgM

Others require CD4+ to trigger rapid proliferation

Undergo complex genetic rearrangements, switching to IgG, IgA or IgE

Further differentiation for plasma cells to produce IgG, IgA, IgE antibody and long-lived memory cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What interaction is needed for CD4+ cells to help B cell differentiation?

A

CD4+ T cells primed by dendritic cells

CD40L:CD40 interaction between T and B cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Complement involved in classical pathway

A

Antibody-antigen immune complex exposes binding site for C1 (acquired immune response)

Triggers activation of cascade; C2, C4

Major amplification step -> C3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Complement involved in mannose binding pathway

A

Activated by direct binding to mannose binding lectin on cell surface
Stimulates classical pathway via C2 and C4 only

Leads to major amplification step via C3 activation

Doesn’t need acquired immune response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Complement involved in alternative pathway

A

Direct trigger of C3 to bacterial cell wall components

Involves factors B, I, P

i. e. lipopolysaccharide of gram negative bacteria
i. e. teichoic acid of gram positive bacteria

Doesn’t need acquired immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What does C3 convertase do in the complement cascade?

A

Triggers formation of membrane attack complex via C5-9

This punches holes in bacterial membranes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Role of cytokines and examples

A

Small protein messengers with immunomodulatory function

Autocrine or paracrine dependent action

E.g.
IL-2, 6, 10, 12
TNF-alpha
TGF-beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Role of chemokines and examples

A

Chemotactic cytokines (attractive) thus directly recruits/hones in leukocytes in inflammatory response

E.g.
CCL19, 21 ligands for CCR7 (direct dendritic cells to lymph nodes)
IL-8, RANTES, MIP-1 alpha and beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How is HIV transmitted?

A

Sexual contact
Infected blood
Mother-to-child (vertical: breastfeeding, in utero, intra partum)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Pathogenesis of HIV

A

RNA retrovirus targets CD4+ T helper cells

Replicates via reverse transcriptase to convert RNA into DNA to go into host cell’s gene

CD4 molecule receptor for HIV and virus binds on other CD4+ cells

Leads to selective loss of CD4+ T cells in immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Which receptors do HIV bind onto on CD4+ T cells?

A 
gp120 = initial binding 
gp41 = conformational change
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Which coreceptors do HIV bind onto on macrophages?

A

CCR5
CXCR4
= both chemokine co-receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Which protein leads to intrastructural support for HIV?

A

Gag protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the innate response to HIV?

A

Non-specific activation of macrophages, NK cells and complement
Stimulation of dendritic cells vita TLR
Release of cytokines and chemokines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the adaptive response to HIV?

A

Neutralising antibodies: anti-gp120, anti-gp41
Non-neutralising antibodies: anti-p24 gag IgG
CD8+ T cells release chemokines: MIP-1a, MIP-1b, RANTES which block co-receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the median time it takes to develop AIDS?

A

Typical progressors: 8-10 years
Rapid progressors: 2-3 years
Long term non progressors: stable and no sx after 10-15 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Screening test for HIV

A

anti-HIV ab via ELISA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Confirmation test for HIV

A

Detect ab via Western Blot after 10 weeks incubation period

= patient needs to be seroconverted; start making abs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is monitored after diagnosis?

A

Viral load via PCR to detect viral RNA

CD4 count via FACS/flow cytometry to assess course of disease

Resistance testing to antiretrovirals

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

How is resistance to antiretrovirals tested?

A

Phenotypic
- viral replication measured in cell cultures under selective pressure of increasing concentrations of antiretroviral drugs compared to wild-type

Genotypic
- mutations determined by direct sequencing of amplified HIV genome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Which drugs affect which part of the HIV life cycle?

A
  1. Attachment/entry
    => attachment inhibitors
    => fusion inhibitors
  2. Reverse transcription and DNA synthesis
    => reverse transcriptase inhibitors
    => NRTI, NNRTI, NrTRI
  3. Integration to host DNA
    => integrase inhibitors
  4. Viral transcription
  5. Viral protein synthesis
  6. Assembly and budding
    => protease inhibitors
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Tx plan for HIV

A

Commence tx immediately after diagnosis

HAART = 2NRTIs + PI (or NNRTI)

  • control viral replication
  • increase CD4 T cell count
  • improve host defence

Pregnancy

  • zidovudine at antepartum IV
  • PO to newborn for 6/52 to reduce transmission
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are the limitations of HAART?

A 
Cannot eradicate latent HIV-1
Fails to restore HIV-specific T-cell responses
Toxicities 
High pill burden 
Adherence 
Threat of drug resistance
Quality of life affected
High costs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Name a fusion inhibitor for HIV and its side effects

A

Enfuvirtide
= local reactions to injections
= hypersensitivity in 0.1-1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Name an attachment inhibitor for HIV

A

Maraviroc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Name some NRTIs for HIV

A 
Nucleoside reverse transcriptase inhibitors
= zidovudine
= didanosine
= stavudine
= abacavir
= emtricitabine
= epzicom
= zalcitabine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Side effects of NRTIs for HIV

A 
Fever
Headache
GI upset
Peripheral neuropathy (zalcitabine, stavudine) 
BMS (zidovudine) 
Mitochondrial toxicity (stavudine) 
Hypersensitivity (abacavir)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Name a nucleotide RTI for HIV and its side effects

A

Tenofovir

= bone and renal toxicity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Name non-RTIs for HIV and their respective side effects

A

Nevirapine: hepatitis and rash
Delavirdine: rash
Efavirenz: CNS effects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Name integration inhibitors for HIV

A

Raltegravir

Elvitegravir

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Name some protease inhibitors for HIV

A 
Indinavir
Nelfinavir
Ritonavir
Fosamprenavir
Lopinavir
Atazanavir
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Name side effects of protease inhibitors for HIV

A

Hyperlipidaemia
Fat redistribution
Type II DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Where are central and effector memory cells found?

A

Central
=> lymph nodes, tonsils
=> found in high endothelial venules (HEVs)

Effector
=> liver, lungs, gut

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Which T cell activation markers are vital for central memory cells?

A

CCR7+ and CD62L high

=> low in effector memory cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What do central and effector memory cells produce?

A

Central
=> IL-2

Effector
=> perforin, IFN-gamma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

T cell memory summary

A

CD45 RO = memory T cells
CD45 RA = naive T cells

CD4 and CD8 remain for long time after infection and proliferate at low rate
Exposure to subsequent antigen -> rapid proliferation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

B cell memory summary

A

B memory cells differentiate into long-lived plasma cells

These cells produce quicker response, more abs, higher affinity abs, more IgG and better abs when B cells exposed to subsequent antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What do CD4+ Th1 cells do?

A

Cell-mediated; help CD8 and macrophages

Produce IL-2, IFN-gamma, TNF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What do CD4+ Th2 cells do?

A

Humoral response; helper T cells

Produce Il-4, IL-5, IL-6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What do CD4+ Th17 cells do?

A

Help neutrophil recruitment

Produce IL-17, IL-21, IL-22

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What passive vaccine do we give for hep A and measles?

A

Human normal Ig = HNIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What passive vaccine do we give for hep B?

A

Hep B immunoglobulin = HBIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What passive vaccine do we give for rabies?

A

Human rabies immunoglobulin = HRIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What passive vaccine do we give for varicella?

A

Varicella zoster immunglobulin = VZIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Which monoclonal antibody do we give for RSV?

A

Paviluzimab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

How do passive vaccines work?

A

Give immunoglobulins to assist with immune response, only lasts for 3 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

How do you do the mantoux test?

A

Inject 0.1ml of 5 tuberculin (purified protein derivative) units intradermally
Examiner arm 48-72 hours later

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

What does a +ve mantoux result mean?

A

+ve = swelling at injection site at least 10mm in diameter

Implies previous exposure to tuberculin protein either previous TB exposure or previous BCG exposure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

What different mechanisms of phagocyte deficiencies are there?

A
  1. Failure to produce neutrophils
    - reticular dysgenesis (production), Kostmann syndrome & cyclic neutropenia (maturation)
  2. Defect of phagocyte migration
    - leukocyte adhesion deficiency
  3. Failure of oxidative killing mechanisms
    - chronic granulomatous disease
  4. Cytokine deficiency
    - IL12, IL12-receptor, IFN-gamma, IFN-gamma receptor deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

What is leukocyte adhesion deficiency characterised by?

A

This is a phagocyte deficiency that results in:

  1. Very high neutrophil counts in blood
  2. Absence of pus formation
  3. Delayed umbilical cord separation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

What mutations results in the following?

a) reticular dysgenesis
b) Kostmann syndrome
c) cyclic neutropenia

A

a) autosomal recessive, mitochondrial metabolism enzyme adenylate kinase 2 (AK2)
b) autosomal recessive, HCLS1-associated protein X-1 (HAX1)
c) autosomal dominant, neutrophil elastase (ELA-2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

What ix are done for chronic granulomatous disease?

A

Nitro-blue tetrazolium test (NBT)

Dihydrorhodamine (DHR) flow cytometry test

Both negative in affected pts as deficient in NADPH oxidase needed to form free radical to give +ve result

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is chronic granulomatous disease characterised by?

A

Absent respiratory burst
Excessive inflammation
Granuloma formation
Lymphadenopathy and hepatosplenomegaly

Susceptible to bacteria, especially catalase +ve, and PLACESS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What are the PLACESS pathogens?

A 
Pseudomonas
Listeria
Aspergillus 
Candida
E. Coli
Staph aureus 
Serratia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Tx for chronic granulomatous disease

A

Interferon gamma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Recurrent infections with NO neutrophils on FBC

A

Kostmann Syndrom

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Recurrent infections with high neutrophils but no abscesses

A

Leukocyte Adhesion Deficienc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Recurrent infections with hepatosplenomegaly and abnormal DHR test (does not fluoresce)

A

Chronic granulomatous disease (CDG)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Infection with atypical mycobacterium, but normal FBC

A

IFN-g Receptor Deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Severe chicken pox, disseminated CMV infection

A

Classical NK Cell Deficiency (recurrent viral infections common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Severe childhood-onset SLE with normal levels of C3 and C4

A

C1q deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Membranoproliferative nephritis and abnormal fat distribution

A

C3 deficiency with nephritic factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Meningococcus meningitis with FHx of sibling dying of the same condition aged 6

A

C7 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Recurrent infections when neutropenic following chemotherapy but previously well

A

MBL deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Characteristics of complement deficiencies

A

Increased susceptibility to bacterial infections, specially encapsulated bacterial infections (NHS)

  1. Neisseria meningitis
  2. Streptococcus pneumonia
  3. Haemophilus influenza
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Why do deficiencies of early classical complement pathway components result in SLE?

A

Complement deficiency results in a deposition of immune complexes due to ineffective promotion of phagocyte-mediated clearance of apoptotic/necrotic cells

This stimulates local inflammation in skin, joints, and kidneys -> SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Which early classical pathways are described in SLE?

A

C1q, C1r, C1s, C2, C4

C2 most common with almost all C2 patients having SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

What is MBL deficiency associated with?

A

Increased infection in immunocompromised patients:

  • premature infants
  • chemotherapy
  • HIV infection
  • antibody deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

How are complement deficiencies diagnosed?

A

CH50 test
- classical pathway test

AP50 test
- alternate pathway test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

C3 +
C4 +
CH50 -
AP50 +

Which complement deficiency?

A

C1q deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

C3 +
C4 +
CH50 +
AP50 -

Which complement deficiency?

A

Factor B deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

C3 +
C4 +
CH50 -
AP50 -

Which complement deficiency?

A

C9 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

C3 +/-
C4 -
CH50 +/-
AP50 +

Which complement deficiency?

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Mx of complement deficiencies

A

Vaccinations
Prophylactic abx
Treat infection aggressively
Screen family members (risk of meningococcal septicaemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Classical NK deficiency

A

Absence of NK cells within peripheral blood

Abnormalities described in GATA2 or MCM4 genes in subtypes 1 and

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

Functional NK deficiency

A

NK cells present but function is abnormal

Abnormality described in FCGR3A gene in subtype

84
Q

What are the mechanisms by which T cell immunodeficiencies can occur?

A

Failure of lymphocyte precursors
- SCID

Failure of thymic development
- DiGeorge syndrome

Failure of expression of HLA molecules
- bare lymphocyte syndromes

Failure of signalling, cytokine production and effector function
- IFN-gamma or receptor deficiency, IL12 or receptor deficiency

85
Q

Severe recurrent infections from 3 months of age, CD4 and CD8 cells are absent, B cells present, immunoglobulin are low. Normal facial features and echocardiogram

A

X-linked SCID

86
Q

Young adult with chronic infection with Mycobacterium marinu

A

IFN-gamma receptor deficiency

87
Q

Recurrent infections in childhood, abnormal facial features, congenital heart disease, normal B cells, low T cells, low IgA and low IgG

A

22q 11.2 deletion syndrome/DiGeorge syndrome

88
Q

6-month old baby with two recent serious bacterial infections. T cells present - but only CD8+. B cells present. IgM present but IgG low

A

Bare lymphocyte syndrome type II

89
Q

What are the mechanisms by which B cell immunodeficiencies can occur?

A

Failure of lymphocyte precursors
- SCID

Failure of B cell maturation
- Bruton’s X-linked hypogammaglobulinaemia

Failure of T cell costimulation
- X-linked hyper IgM syndrome

Failure of production of IgG antibodies
- common variable immune deficiency, selective antibody deficiency

Failure of IgA production
- selective IgA deficiency

90
Q

Adult with bronchiectasis, recurrent sinusitis and development of atypical SLE

A

Common variable immunodeficiency

91
Q

Recurrent bacterial infections as a child, episode of PCP, high IgM, absent IgA and absent IgG

A

X-linked hyper IgM syndrome due to CD40L mutation

92
Q

1-year old boy. Recurrent bacterial infections. CD4 and CD8 T cells present. B cells absent. IgG, IgA and IgM absent

A

Bruton’s X-linked hypogammaglobulinaemia

93
Q

Recurrent respiratory tract infections, absent IgA, normal IgM and normal IgG

A

IgA deficiency

94
Q

Characteristics of SCID

A 
Unwell by 3 months of age
Infections of all types
Failure to thrive
Persistent diarrhoea 
Poorly developed lymphoid tissues (germinal centres) and thymus 
Family history of early infant death
95
Q

Most common mutation of SCID

A

X-linked SCID

Mutation of gamma chain of IL2 receptor on chromosome Xq13.1

96
Q

What is ADA deficiency?

A

16.5% of all SCID

Adenosine deaminase deficiency (autosomal recessive), required for lymphocyte cell metabolism so phenotype is low in everything (T, B, NK cells)

97
Q

What protects the SCID neonate in the first 3 months of life?

A

Maternal IgG high present in colostrum first 3 months of life

Eventually diminishes over time, however, SCID baby does not make neonatal IgG

98
Q

Characteristics of DiGeorge Syndrome

A

CATCH-22

Cardiac abnormalities (TOF)
Abnormal facies (high forehead, low set ears)
Thymic aplasia (T cell lymphopenia)
Cleft palate
Hypocalcaemia/hypoparathyroidism 
22- chromosome affected
99
Q

What mutation causes DiGeorge syndrome?

A

Deletion at 22q11.2

100
Q

What causes BLS type II?

A

Bare lymphocyte syndrome type II

Defect in one of the regulatory proteins involved in class II gene expression

101
Q

Clinical features of T cell deficiencies

A 
Viral infections (CMV)
Fungal infections (pneumocystis, cryptosporidium)
Some bacterial (intracellular - myco TB, Salmonella)
Early malignancy
102
Q

How are lymphocyte deficiencies diagnosed?

A
  1. WCC
  2. Lymphocyte subsets
  3. Serum immunoglobulins (if CD4 deficient, IgG surrogate marker for function) & protein electrophoresis
  4. Functional tests
  5. HIV
103
Q 
CD4 T cell -
CD8 T cell - 
B cell +/-
IgM +/-
IgG -

Which T cell deficiency is this?

104
Q 
CD4 T cell -
CD8 T cell - 
B cell +
IgM +
IgG +/-

Which T cell deficiency is this?

105
Q 
CD4 T cell -
CD8 T cell + 
B cell +
IgM +
IgG -

Which T cell deficiency is this?

A

Bare lymphocyte syndrome type II

106
Q

Mx of T cell deficiencies

A 
Infection prophylaxis & treatment
Ig replacement
Haematopoietic stem cell transplantation 
Gene therapy (experimental) 
Thymic transplant in DiGeorge syndrome
107
Q

Mx of B cell deficiencies

A

Aggressive tx of infection
If replacement every 3 weeks (pooled plasma w diverse IgG
Bone marrow transplant
Imms in selective IgA deficiency (not effective if no IgG)

108
Q

What is WAS?

A

Wiskott-Aldrich syndrome

X-linked recessive, mutation in WAS gene, needed for T cell-APC interaction

Results in low platelets, IgM, lymphocytes

Results in increased IgA, IgE and risk of malignant lymphoma

109
Q

What causes Bruton’s X-linked hypogammaglobulinaemia?

A

Abnormal B cell tyrosine kinase (BTK) gene

Results in no mature B cells in circulation thus no antibody production

110
Q

Clinical characteristics of Bruton’s X-linked hypogammaglobulinaemia

A

Only boys
Recurrent infections during childhood
Absent/scanty lymph nodea and tonsils (primary follicles and germinal centres absent)
Failure to thrive

111
Q

What causes hyper IgM syndrome?

A

Mutation in CD40 ligand gene

Results in CD4+ T cells UNABLE to signal to B cells thus B cells unable to enter germinal centre reaction to undergo isotype switching and affinity maturation

112
Q

Clinical characteristics of hyper IgM syndrome

A 
Boys present with failure to thrive in first few years of life 
Recurrent bacterial infections
Pneumocystis jiroveci infection
Autoimmune disease
Malignancy
113
Q

What is common variable immune deficiency defined by?

A

Marked reduction in IgG with low IgA/IgM

Poor/absent response to immunisation

Absence of other defined immunodeficiency

114
Q

Clinical features of common variable immune deficiency

A

Recurrent bacterial infections with severe end-organ damage
- Pneumonia, persistent sinusisit, gastroenteritis

Pulmonary
- bronchiectasis, ILD

GI
- IBD, sprue like illness bacterial overgrowth

Autoimmune
- AIHA, RA, pernicious anaemia, thyroiditis, vitiligo

Malignany
- non-Hodgkin lymphoma

115
Q

Clinical features of antibody deficiency (or CD4 T cell deficiency)

A

Bacterial infections (Staph, Strep)

Toxins (tetanus, diptheria)

Some viral infections (enterovirus)

116
Q 
CD4 T cell +
CD8 T cell + 
B cell - 
IgM - 
IgG -
IgA -

Which B cell deficiency is this?

A

Bruton’s X-linked hypogammaglobulinaemia

117
Q 
CD4 T cell +
CD8 T cell + 
B cell + 
IgM ++
IgG -
IgA -

Which B cell deficiency is this?

A

Hyper IgM X-linked

118
Q 
CD4 T cell +
CD8 T cell + 
B cell +
IgM +
IgG +
IgA -

Which B cell deficiency is this?

A

Selective IgA deficiency

119
Q 
CD4 T cell +
CD8 T cell + 
B cell +
IgM +
IgG -
IgA -

Which B cell deficiency is this?

A

Common variable immune deficiency

120
Q

Immunopathology in absence of infection possibilities

A

Autoinflammatory
Mixed
Autoimmunity

121
Q

Periodic fever lasting 48-96 hours with abdo pain, chest pain, rash and arthralgia

Mutation found in MEFV gene

A

Familial Mediterranean fever

  • gene encodes for pyrin-marenostrin
  • needed for activation of neutrophils
122
Q

Long term risk of Familial Mediterranean fever

A

AA amyloidosis

  • kidney, liver, spleen
  • nephrotic syndrome -> renal failure
123
Q

Tx of MEFV gene mutation condition

A

Familial Mediterranean Fever

  • colchicine (affects neutrophils)
  • anakinra (IL-1 R antagonist)
  • etanercept (TNF-alpha inhibitor)
124
Q

Defect in AIRE leading to hypoparathyroidism, Addison’s, hypothyroidism, diabetes, vitiligo, enteropathy condition

A

Auto-immune polyendocrine syndrome type 1 (APS1)

Auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED)

125
Q

Defect in Foxp3 leading to diabetes mellitus, hypothyroidism, enteropathy condition

A

Immune dysregulation polyendocrinopathy enteropathy (IPEX)

126
Q

Defect in FAS pathway leading to high lymphocyte with large spleen and lymph nodes leading to autoimmune cytopenias and lymphomas

A

Autoimmune lymphoproliferative syndrome (ALPS)

127
Q

IBD1 gene on chromosome 16 identified as NOD2 (CARD-15) is associated with which condition?

128
Q

Which gene is associated with Crohn’s?

A

IBD1 gene on chromosome 16 identified as NOD2 (CARD-15)

129
Q

The genetic polymorphism PTPN22 is associated with which conditions?

A

Rheumatoid arthritis
SLE
T1DM

130
Q

The genetic polymorphism CTLA4 is associated with which conditions?

A

SLE
T1DM
Autoimmune thyroid disease

131
Q

HLA-B27 associated condition

A

Ankylosing spondylitis

132
Q

HLA DR15/DR2 associated condition

A

Goodpasture’s syndrome

133
Q

HLA DR3 associated conditions

A

Graves disease
SLE
T1DM

134
Q

HLA DR4 associated conditions

A

Rheumatoid arthritis

T1DM

135
Q

Type 1 hypersensitivity diseases

A 
Atopic dermatitis (infantile eczema)
Food allergy
Oral allergy syndrome
Latex food syndrome
Allergic rhinitis
Acute urticaria
136
Q

Which food allergies to latex allergy sufferers have typically?

A 
Chestnut
Avocado
Banani
Potato
Tomato
Kiwi
Papaya
Eggplant
Mango
Wheat 
Melon
137
Q

Common Ig-E mediated mast cell degranulation triggers

A

Peanut
Penicillin
Stings
Latex

138
Q

Common non Ig-E mediated mast cell degranulation triggers

A

NSAIDs
IV contrast
Opioids
Exercise

139
Q

Anaphylaxis mx

A 
Elevate legs
100% oxygen
IM adrenaline 500 mcg
Inhaled bronchodilators 
Hydrocortisone 200mg IV
Chlorphenamine 10mg IV
IV fluids 
Seek help
140
Q

Ix in allergy

A

Skin prick tests
Quantitative specific IgE to putative allergen (RAST)
Component-resolved diagnostics
Challenge Test
Measure mast cell tryptase during acute episode (lol)

141
Q

Which mediators are indicated in the following hypersensitivity disorders?

a) Type 1
b) Type 2
c) Type 3
d) Type 4

A

a) Ig-E - mast cell degranulation
b) IgG or IgM antibody - reacts with cell/matrix associated self-antigen
c) IgG or IgM immune complex - Ab vs soluble Ab mediated tissue damage
d) T-cell mediated - delayed hypersensitivity

142
Q

Type 2 hypersensitivity diseases

A 
Haemolytic disease of newborn 
Autoimmune haemolytic anaemia 
Autoimmune thrombocytopenic purpura
Goodpasture's syndrome
Pemphigus vulgaris
Graves disease
Myasthenia Gravis
Acute rheumatic fever
Pernicious anaemia
Churg-Strauss syndrome (eGPA)
Wegener's granulomatosis (GPA) 
Microscopic polyangiitis (MPA) 
Chronic urticaria
143
Q

Type 3 hypersensitivity diseases

A

Mixed essential cryglobulinaemia
Serum sickness
Polyarteritis nodosa (PAN )
Systemic lupus erythematosis (SLE)

144
Q

What is Evan’s syndrome?

A

Autoimmune haemolytic anaemia + ITP

sad times

145
Q

Type 4 hypersensitivity diseases

A 
T1DM
Multiple sclerosis 
Rheumatoid arthritis
Contact dermatitis
Mantoux test
Crohn's disease
146
Q

CREST syndrome

A 
Limited cutaneous scleroderma: 
Calcinosis
Raynaud's 
oEsophageal dysmotility 
Sclerodactyly 
Telangiectasia
147
Q

Sx seen in diffuse cutaneous scleroderma

A

CREST
GIT
Interstitial pulmonary disease
Renal problems

148
Q

42 year old lady presents with dry mouth and eyes, swelling on the sides of her face and issues with her kidneys

What antibodies do you expect to be present?

A

Anti-Ro and anti-La

= Sjorgen’s syndrome

149
Q

Which skin condition is seen with coeliac disease?

A

Dermatitis herpetiformis

150
Q

What autoantibodies are seen in antiphospholipid syndrome?

A

anti-cardiolipin
anti-beta-2-glycoportein
anti-lupus anticoagulant

151
Q

What autoantibodies are seen in autoimmune hepatitis?

A

anti-smooth muscle
anti-liver kidney microsomal-1 (anti-LKM-1)
anti-soluble liver antigen (anti-SLA)

152
Q

What autoantibodies are seen in autoimmune haemolytic anaemia?

A

anti-Rh blood group antigen

153
Q

What autoantibodies are seen in autoimmune thrombocytopenic purpura?

A

anti-glycoprotein IIb-IIIa

154
Q

Which conditions would you see p-ANCA in?

A 
Churg-Strauss syndrome (eGPA)
Microscopic polyangiitis (MPA)
155
Q

What autoantibodies are seen in coeliac disease?

A

anti-tissue transglutaminase

anti-endomysial

156
Q

What autoantibodies are seen in congenital heart block in infants of mothers with SLE?

157
Q

What autoantibodies are seen in dermatitis herpetiformis?

A

anti-endomysial

158
Q

What autoantibodies are seen in dermatomyositis?

A

anti-Jo-1 (t-RNA synthetase)

159
Q

What autoantibodies are seen in diffuse cutaneous scleroderma?

A

anti-topoisomerase/Scl70
anti-RNA Pol I, II, III
anti-fibrillarin

160
Q

What autoantibodies are seen in Goodpasture’s syndrome?

161
Q

What autoantibodies are seen in Graves disease?

A

anti-TSH receptor

162
Q

What autoantibodies are seen in Hashimoto’s thyroiditis

A

anti-thyroglobulin

anti-thyroperoxidase

163
Q

What autoantibodies are seen in limited cutaneous scleroderma?

A

anti-centromere

164
Q

What autoantibodies are seen in mixed connective tissue diease?

A

anti-U1RNP

165
Q

What autoantibodies are seen in pernicious anaemia?

A

anti-parietal

anti-intrinsic factor

166
Q

What autoantibodies are seen in myasthenia gravis?

A

anti-Ach receptor

167
Q

What autoantibodies are seen in polymyositis?

168
Q

What autoantibodies are seen in primary biliary cirrhosis?

A

anti-mitochondrial

169
Q

What autoantibodies are seen in rheumatoid arthritis?

A

anti-cyclic citrullinated peptide

anti-RF (less specific)

170
Q

What autoantibodies are seen in systemic lupus erythematosis?

A

anti-dsDNA

anti-Ro, La, Sm, U1RNP

171
Q

What autoantibodies are seen in T1DM?

A

anti-glutamate decarboxylase

anti-pancreatic beta cells

172
Q

What autoantibodies are seen in Wegener’s granulomatosis (GPA)?

173
Q

What actions boost the immune response?

A
  1. Vaccination
  2. Replacement of missing components
  3. Cytokine therapy
  4. Blocking immune checkpoints - for
    advanced melanoma
174
Q

What suppresses the immune response?

A
  1. Steroids
  2. Anti-proliferative agents
  3. Plasmapheresis
  4. Inhibitors of cell signaling
  5. Agents directed at cell surface antigens
  6. Agents directed at cytokines
175
Q

Why doesn’t vaccination work effectively in the elderly?

A

Immune senescence: Increased frequency of terminally differentiated effector memory T cells in
the elderly; Increased expression of senescence markers; Much reduced production of recent
thymic emigrants which drive the naïve T-cell repertoire.

Nutrition: insufficient energy because of poor nutrition; Reduced availability of trace elements and
minerals (reduced gut absorption)

176
Q

Drugs that block immune checkpoints for advanced melanoma

A

Ipilimumab
Pembrolizumab
Nivolumab

177
Q

Side effects of immunosuppresives therapies

A 
Chronic infection
- TB, HIV, Hep B, C, JCV
Malignancy
- lymphoma, melanoma, HPV
Autoimmunity
- SLE, vasculitis, AIH
178
Q

Injection site reactions

A

Peak reaction at ~48 hours
May also occur at previous injection sites (recall reactions)
Mixed cellular infiltrates, often with CD8 T cells
Not generally IgE or immune complexes

179
Q

Infusion Reactions

A

Urticaria, hypotension, tachycardia, wheeze – IgE mediated
Headaches, fevers, myalgias – not classical type I hypersensitivity
Cytokine storm

180
Q

Use of human normal immunoglobulin (IVIG)

A
  1. primary and secondary immunodeficiency
  2. ITP
  3. MG
  4. GBS
  5. Kawasaki disease
  6. toxic epidermal necrolysis
  7. pneumonitis induced by CMV following transplantation
  8. low serum IgG levels following haematopoietic stem cell transplant for malignancy
  9. dermatomyositis
  10. chronic inflammatory demyelinating polyradiculopathy
181
Q

Anti-proliferative agent action

A

Inhibit DNA synthesis with cells with most rapid turnover most sensitive to it

182
Q

Anti-proliferative agent examples

A

Alkylates:
Cyclophosphamide

Anti-metabolites:
Mycophenolate
Mofetil
Azathioprine

Anti-folate:
Methotrexate

183
Q

Calcineurin inhibitors used in prophylaxis for transplant pts

A

Tacrolimus
Cyclosporin

Inhibits cell signaling

184
Q

IL-2 inhibitors used in prophylaxis for transplant pts

185
Q

Anti-CD20 agent used against lymphoma, RA, and SLE

186
Q

Anti-CD25 agent used in allograft rejection

A

Basiliximab

187
Q

IL-2 receptor antibody that targets CD25 used in organ transplant rejection prophlyaxis

A

Daclizumab

188
Q

anti-TNF alpha agents

A

Infliximab
Adalimumab
Certolizumab
Golimumab

189
Q

Which conditions require anti-TNF alpha agents?

A 
Rheumatoid arthritis, 
Ankylosing spondylitis, 
Psoriasis, psoriatic 
arthritis, Inflammatory 
bowel disease
190
Q

What is denosumab and when is it used?

A

anti-RANK ligand antibody

Osteoporosis, multiple myeloma, bone metastases

191
Q

How does recognition in the immune system occur?

A

Immune Recognition:

  • T-Cells (TCs) recognise antigen with MHCs on APCs
  • B-Cells (BCs) can recognise just antigen
192
Q

Most important HLA classes in transplants

A

DR > B > A

193
Q

Types of recognition in tranplants

A
  1. Direct
    Donor APC presenting antigen and/or MHC to recipient T-cells. Acute rejection mainly involves
    direct presentation.
  2. Indirect
    Recipient APC presenting donor antigen to recipient T-cells – i.e. the immune system working
    normally, as it would for an infection. Chronic rejection mainly involves indirect presentation.
194
Q

Phases of immune response to transplant

A

Phase 1: recognition of foreign antigens
Phase 2: activation of antigen-specific lymphocytes;
proliferation and maturation of B cells with Ab production
Phase 3: effector phase of graft rejection

Involves CD4+ cells, cytotoxic T cells, macrophages, Abs binding to graft endothelium

195
Q

How should acute transplant rejections be treated?

A

Cellular – Steroids, OKT3/ATG

Ab-mediated – IVIG, plasma exchange, anti-C5, anti-CD20

196
Q

GVHD prophylaxis

A

Methotrexate/cyclosporine

197
Q

GVHD treatment

A

Corticosteroids

198
Q

Post-transplantation complications

A

Infection
- CMV, BK virus, Pneumocystis carinii

Malignancy
- Kaposi’s, EBV, melanoma

Atherosclerosis
- HTN, hyperlipidaemia, MI

199
Q

What are the pathogenic antibodies in bullous pemphigoid?

A

Anti-Hemidesmosome

200
Q

What is the target of the antibody p-ANCA?

A

Myeloperoxidase

201
Q

What is the target of the antibody c-ANCA?

A

Proteinase-3

202
Q

Which vaccines are avoided in pregnancy and immunocompromised patients?

A

Live attenuated

203
Q

What type of vaccines are typically used for viruses?

A

Subunit (recombinant)

204
Q

What type of vaccines are typically used for encapsulated bacteria?

205
Q

Which vaccines include pathogens that have been rendered inert - usually by heat killing or formaldehyde?

A

Inactivated

206
Q

Which primary immunodeficiency is diagnosed with Nitroblue-tetrazolium (NBT) or dihydrorhodamine (DHR) tests?

A

Chronic granulomatous disease

207
Q

Mutation in what cell surface receptor may confer immunity from HIV?

Year 5 Pathology (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions