Immuno 1 Flashcards

1
Q

Cells of the innate immune system

A

Polymorphic - Neutrophils, eosinophils, basophils
Monocytes/Macrophages
Natural Killer Cells
Dendritic Cells

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2
Q

Soluble components of the innate immune system

A

Complement
Acute phase proteins
Cytokines/Chemokines

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3
Q

Fc Receptors

A

Polymorphic cells of innate system use Fc receptors to recognise pathogenic antigens

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4
Q

Dendritic Cells

A

Present antigens to lymph nodes

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5
Q

NK Cells

A

Destroy cells not expressing HLA 1

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6
Q

Oxidative Killing

A

NADPH catalyses formation of reactive oxygen species

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7
Q

Non-Oxidative Killing

A

Lysosome action

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8
Q

Macrophage action

A

Phagocytose pathogens and present processed antigens to T cells to activate adaptive response

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9
Q

Complement - Classical pathway

A

Activated by Ag-Ab Complexes

C1,2,3 activates C3

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10
Q

Complement - Mannose Binding Lectin

A

Recognises microbial cell surface carbohydrates (mannose)
C4,C2
Activates C3

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11
Q

Complement - Alternative Pathway

A

Recognises bacterial cell wall

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12
Q

Complement - Common Pathway

A

C3 activates C5-C9 which form the Membrane Attack Complex

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13
Q

Components of the adaptive immune response

A

B Cells - humoral
T Cells - Cellular
Cytokines
Chemokines

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14
Q

T cell classification

A

All have CD3
CD4 - Helper T Cells
CD8 - Cytotoxic T Cells

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15
Q

Primary Lymphoid Organs

A

Bone Marrow

Thymus

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16
Q

Secondary Lymphoid Organs

A

Spleen
Lymph Nodes
Mucosal Associated Lymphoid Tissue

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17
Q

T Cell Selection

A

Positive Selection -> Intermediate affinity for HLA, no affinity/too high affinity are destroyed

Negative Selection -> Cells with affinity for tissue self antigens are destroyed

Affinity for HLA I -> CD8+
Affinity for HLA II -> CD4+

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18
Q

B Cell selection

A

No recognition of self in bone marrow

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19
Q

B Cell proliferation

A

T Cell-Independent = B Cells secrete IgM

Germinal Centre Reaction = Dendritic Cells prime CD4+ T cells which present antigens to B cell, leading to proliferation, somatic hypermutation and isotype switching to IgG, A, E

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20
Q

Types of primary immunodeficiencies

A

Innate
Adaptive
Complement

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21
Q

Deficiencies in neutrophil production

A

Kostmann Syndrome
Cyclic Neutropenia
Treatment = G-CSF (Granulocyte colony stimulating factor)

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22
Q

Kostmann’s Syndrome

A

Autosomal Recessive
HAX 1 Protein
G-CSF

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23
Q

Cyclic Neutropenia

A

Autosomal Dominant
Episodic Neutropenia every 4-6 Weeks
Mutation in ELA 2
G-CSF

24
Q

Deficiencies in Leukocyte migration

A

Leukocyte Adhesion Deficiency

25
Q

Leukocyte Adhesion Deficiency

A

CD11a/18 is usually expressed on neutrophils, but is deficient here. usually binds ICAM-1 on endothelial cells

Neutrophils cannot exit bloodstream, hence high neutrophil count, no pus formation

Delayed Umbilical Cord Sloughing

26
Q

Leukocyte Adhesion Deficiency Treatment

A

Haematopoietic Stem Cell transplant

27
Q

Deficiencies in Oxidative Killing

A

Chronic Granulomatous Disease

28
Q

Chronic Granulomatous Disease

A

NAPDH Oxidase deficiency leads to failure to produce reactive oxygen species = impaired killing

Granuloma Formation
Lymphadenopathy
Hepatosplenomegaly
Lots of Pus

Susceptible to catalase positive organisms

29
Q

Chronic Granulomatous Disease treatment

A

IFN-y

30
Q

Chronic Granulomatous Disease infections

A

Catalase positive organisms (since these are killed by oxidative killing)

Pseudomonas
Listeria
Aspergillus
Candida
E Coli
Staph A 
Serratia
31
Q

Test for chronic granulomatous disease

A

Nitroblue Tetrazolium
Hydrogen Peroxide = yellow to blue = normal

Dihydrhodamine Flow Cytometry test
DHR to Fluorescent rhodamine in presence of hydrogen peroxide. Fluorescent = normal

32
Q

Cytokine Deficiency

A

IL12, IL12R, IFNy, IFNyR deficiency
Usually needed for macrophage-T cell communication

Infection with intracellular organisms such as mycobacteria and salmonella

33
Q

T Cell - Macrophage Communication

A

Macrophages produce IL-12, which stimulate T Cells to produce IFN-y, which induce TNF activation and formation of free radicals

34
Q

Classical Pathway Deficiencies

A

C1q/r/s, C2, C4 deficiency
C2 = common = SLE
Severe skin disease
Lots of infections

35
Q

MBL pathway deficiencies

A

30% heterozygous
6-10% no MBL
Increased risk of infection if other immune impairment, ie. prematurity, chemo, HIV, Ab deficiency

36
Q

Common Pathway Deficiency

A
C3, C5-9 
Risk of 
N. meningitidis *
S. pneumoniae
H. influenzae

Low CH50 and low AP50

37
Q

Why does SLE cause complement deficiency?

A

Lupus causes production of Ab-Ag immune complexes

These consume C3/C4 = low complement, despite there not being a primary complement deficiency

38
Q

Why do nephritic factors cause complement deficiency?

A

Auto-Abs against complement associated with glomerulonephritis and partial lipodystrophy

39
Q

Complement deficiency investigations

A

C3 levels
C4 levels
CH50 (classical) levels
AP50 (alternative) levels

40
Q

Management of Complement Deficiency

A

Screening family members
Prophylactic Abx
Vaccination against encapsulated bacteria
Aggressive treatment of active infections

41
Q

Alternative Pathway Deficiencies

A

Factors B, I, P

Infections with encapsulated bacteria

42
Q

Active vs Severe SLE complement levels

A
Active = Low C4, Normal C3
Severe = Low C3, Low C4
43
Q

Lymphoid pregenitors deficiency

A

Severe Combined Immunodeficiency
Reticular Dysgenesis
X Linked SCID
ADA Deficiency

44
Q

Reticular Dysgenesis

A

Autosomal Recessive

Adenylate Kinase 2 mutation = 0 production of lymphocytes, neutrophils, monocytes, platelets

Most severe SCID

45
Q

X-Linked Severe Combined Immunodeficiency Disorder

A

y-chain IL-2 mutation

Poor cytokine response

No T cells, NK cells, immature B cells

Only boys

46
Q

Adenosine Deaminase Deficiency

A

Needed for lymphocyte metabolism

Low B,T,NK

Peg-ADA

47
Q

Lymphoid pregenitor deficiency management

A

Stem Cell transplant

48
Q

T Cell maturation deficiency

A

Di George Syndrome

49
Q

Di George Syndrome

A

Defect of development of pharyngeal pouch
22q11.2 Deletion

CATCH 22

Cardiac abnormalities (TOF)
Abnormal facies
Thymic aplasia*
Cleft palate
Hypocalcaemia

22 = chromosome

50
Q

Di George Investigation findings

A
Low T
Normal B 
Low IgG
Better with age
22q11.2 deletion
51
Q

Di George Management

A

Thymic Transplant (to quadriceps)

52
Q

Deficiency due to CD4/8 T cell selection

A

Bare lymphocyte syndrome
MHC gene expression defect

Class I = Type I = no CD8
Class II = Type II = No CD 4 = Low IgG, IgA, IgE

53
Q

Deficiency in B Cell development

A

Bruton’s X-Linked a-gammaglobulinaemia

Defect in B cell tyrosine Kinase gene
B cells cannot mature
No IgG after 3 months
No lymph nodes/tonsils

IV Ig treatment

54
Q

immunoglobulin defects

A

Selective IgA Deficiency

Combined Variable Immune Deficiency

55
Q

Selective IgA Deficiency

A

1:600
2/3 asymptomatic
1/3 recurrent resp tract infections (IgA = mucosal membranes)

56
Q

Combined Variable Immune Deficiency

A

Low IgA,G,E
Resp + GI Infections
Autoimmune disease, granulomatous disease

57
Q

Defect in class switching

A

Hyper IgM Syndrome
CD40-L defect in T cells on Xq26 = T cell defect leading to no T cell stimulation of B Cell maturation

= no class switching = high IgM, no others