Immunology Flashcards

1
Q

Bordetella Pertussis

A

Whooping cough

Gram negative

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2
Q

Primary Antibody Deficiency associated with sinusitis and ottis media

A

Selective IgA deficiency
Common variable immundeficieny
Specific antibody deficiency
X linked agammaglobulinemia

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3
Q

Complement system disorders

A

Patients with laryngeal angioedema

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4
Q

Severe congenital Neutropenia

Kostman Syndrome

A

Rare genetic disorder
Accumulation of precursor cells in bone marrow
Recurrent bacterial/fungal infections

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5
Q

Leukocyte adhesion deficiency

A

Caused by defect in CD18 integrin gene

Failure of neutrophil adhesion and migration

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6
Q

Chronic granulomatous disease

A

Deficiency of intracellular killing mechanisms of phagocytes - absent respiratory burst
Impaired killing of intracellular microorganisms

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7
Q

How can adaptive immune system go wrong?

A

Defects in leukocyte development

Defects in leukocyte effector functions

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8
Q

DiGeorge syndrome

A

Failure of production of CD4+ and CD8+ T cells
Developmental defect of pharyngeal pouch
Low set folded ears, high forehead, cleft pallet, small mouth and jaw
Hypocalcaemia
Oesophageal atresia

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9
Q

X linked agammaglobulinaemia

A

Failure to produce b cells

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10
Q

Hyper IgM syndrome

A

Severe reduction in serum IgG and IgA

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11
Q

Clinical features of T cell deficiencies

A

Recurrent infections
Opportunistic infections
Malignancies at a young age
Autoimmune disease

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12
Q

Clinical features of b cell deficiencies

A

Recurrent infections
Opportunist infections
Antibody mediated autoimmune disease

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13
Q

Hypersensitivity

A

Immune response resulting in damage to bystander
Usually exaggeration of normal immune mechanism
Pathophysiological basis for chronic diseases (allergy and autoimmunity)

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14
Q

Gel and Coomb’s Classification

A
  1. Immediate Hypersensitivity
  2. Direct cell effects
  3. Immune Complex Mediated
  4. Delayed Hypersensitivity
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15
Q

Type 1

A

Immediate Hypersensitivity
Allergy
IgE mediated antibody response to external antigen

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16
Q

2 stages of allergic disease

A
  1. Sensitisation

2. Allergic

17
Q

Sensitisation stage

A

Mast cells and eosinophils express receptors for IgE antibody
First encounter allergen, B cells produce antigen specific IgE antibody
Allergen is cleared
Residual IgE antibodies bind to circulating mast cells

18
Q

Allergic stage

A

Re-encounter antigen
Allergen binds to IgE-coated mast cells and disrupt cell membrane
Release of vasoactive mediators (histamine)
Increased expression of pro-inflammatory cytokines and leukotrines

19
Q

Clinical features of allergic disease

A

Muscle spasm
Mucosal inflammation
Inflammatory Cell infiltrate

20
Q

Type 2

A

Direct Cell Effects
Cell surface antigens
Involve IgM or IgG
Normal adaptive immune response

21
Q

Goodpastures Syndrome

A

Type 2 Hypersensitivity
Autoimmune disease affecting lung and kidneys
Presence of auto-reactive antibodies to the alpha 3 chain of type IV collagen present in basement membranes of alveoli
From environmental insult

22
Q

Treatment of Goodpastures Syndrome

A

Corticosteroids
Plasmapheresis
STOP SMOKING

23
Q

Type 3

A

Immune Complex Mediated
Soluble Antigens
In presence of excess antigen, antibody binds forming small immune complexes
Trapped in small blood vessels, joints and glomeruli
Activation of complement system, opsonisation and neutrophils and macrophages

24
Q

Acute Hypersensitivity Pneumonitis

A

TYPE 3 HYPERSENSITIVTY
Inhaled antigens deposited in lungs
Stimulate antibody formation
Antibodies form immune complexes with antigen
Complement activation, inflammation etc
Leukocyte accumulation and inflammation within alveoli

25
Q

Type IV

A

Delayed Type Hypersensitivity

TB and Sarcoidosis

26
Q

Sarcoidosis

A

Multi-system granulomatous disease
Small patches of red and swollen tissue to develop in organs
Affects lungs and skin

27
Q

Management of sarcoidosis

A

Wait
NSAIDS
Systemic Corticosteroids

28
Q

Autoimmunity

A

Presence of immune responses against self-tissue/cells

29
Q

How does immune system deal with presence of auto-reactive T cells and B cells

A

Self tolerance mechanisms:

  1. Deletion of self-reactive lymphocytes in primary lymphoid tissues (central tolerance)
  2. Inactivation of self -reactive lymphocytes in peripheral tissues that escape central tolerane
30
Q

Regulatory T cells

A

Essential in suppressing hyper-reactive or auto-reactive T cells
Via production of anti-inflammatory cytokines

31
Q

Pathogenesis of autoimmune disease

A

Genetic Susceptibility
Breakdown of immune tolerance to self-antigens
Autoimmune disease

32
Q

IPEX Syndrome

A
Monogenic Disorder (Single gene defect causes autoimmune disease)
Symptoms = severe infections, excess etc
Treatment = hematopoeietic stem cell transplant,
Immunosuppressive drugs, parenteral nutrition
33
Q

Pathogenesis of IPEX syndrome

A

X linked
Mutation in FOXP3 gene, needed for development of regulatory T cells
IPEX = failure of peripheral tolerance mechanisms due to defective T cells

34
Q

HLA genes

A

Highly polymorphic

35
Q

Graves disease

A

Hyperthyroidism

Auto-antibodies generated that bind to thyroid stimulating hormone receptor

36
Q

Systemic Lupus Erythematosus

A

Type III
Severe relapsing autoimmune disease
Increased apoptosis, defective clearance of apoptotic material and dysregulation of neutrophil NETs

37
Q

Rheumatoid Arthritis

A

Type IV

Complication = inflamamtion in lungs and heart