Immunopathology Flashcards

1
Q

DiGeorge Syndrome

A

No Development of third and fourth pharyngeal pouches
22q11 microdeletion
T-cell deficiency–no thymus
Hypocalcemia– lack parathyroids
Abnormalities of heart/great vessels/face

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2
Q

Severe Combined immunodeficiency

A
Defective cell-medicated and humoral immunity
Cytokine receptor defects
Adenosine deaminase (ADA) deficiency-- build up of adenosine and deoxyadenosine
MHC class II deficiency--necessary for CD4 helper T cell activation and cytokine production
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3
Q

X-Linked Agammaglobulinemia

A

Complete lack of immunoglobulin due to disordered B-cell maturation

Mutated Bruton tyrosine kinase

Presents after 6 months of life due to antibodies present during first 6 months from mother for protection

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4
Q

Common variable immunodeficiency

A

Low immunoglobulin due to B-cell or helper T-cell defects
B-cells- Igs
T-cells- IL-4 and 5

Increased risk for autoimmune dz and lymphoma

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5
Q

IgA deficiency

A

Low serum and mucosal IgA

increased risk for mucosal infection–especially viral

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6
Q

Wiskott-Aldrich syndrome

A

Thrombocytopenia/eczema/recurrent infections

Bleeding is a major cause of death

Mutation in the WASP gene

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7
Q

Complement deficiencies

A

C5-C9–increased risk for Neisseria infection

C1 inhibitor- angioedema–edema of the skin and mucosal surfaces

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8
Q

Hyper-IgM syndrome

A

Elevated IgM

Mutated CD40L (On helper T cell) or CD40 receptor (on B cells)

B-cells cannot be activated–cytokines for class switching are not pro ducted (IL-4 and 5)

Low IgA/IgG/IgE– result in recurrent pyogenic infections–poor opsonization

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9
Q

What activates Caspase-1 and what does this lead to?

A

NLRs which cause Caspase-1 to release IL-1 and IL-18

This attracts immune cells to site of infection and IL-1 causes Fever

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10
Q

What role does IL-6 play in innate immunity?

A

increase synthesis and release of hepcidin which decreases reabsorption of Fe in duodenum and prevents release from macrophages—-gets Fe away from bacteria

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11
Q

What is Type I immediate hypersensitivity?

A

Triggered by binding of an antigen to IgE antibody on surface of Mast cell/basophil

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12
Q

What is Type II hypersensitivity?

A

Antibody directed against antigens on cell membrane or in ECM

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13
Q

What is autoimmune hemolytic anemia?

A

Type II hypersensitivity

  • -Target–RBC membrane protein
  • -hemolysis and anemia
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14
Q

What is autoimmune thrombocytopenia purpura?

A

Type II hypersensitivity

  • -Target– Platelet membrane proteins
  • -bleeding
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15
Q

What is Pemphigue vulgaris?

A

Type II hypersensitivity

  • -target– proteins in intercellular junctions
  • -Skin vesicles
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16
Q

What is Vasculitis caused by ANCA?

A

Type II hypersensitivity

  • -target–Neutrophil granule proteins
  • -Vasculitis
17
Q

What is Goodpasture syndrome?

A

Type II hypersensitivity

  • -target– Noncollagenous protein in BMs of kidney glomeruli and lung alveoli
  • -Nephritis/lung hemorrhage
18
Q

What is acute rheumatic fever?

A

Type II hypersensitivity

  • -target–streptococcal cell wall antigen–antibody cross-reacts with myocardial antigen
  • -myocarditis/arthritis
19
Q

What is Myasthenia gravis?

A

Type II hypersensitivity

  • -target–acetylcholine receptor
  • -muscle weakness/paralysis
20
Q

What is graves dz?

A

Type II hypersensitivity

  • -target–TSH receptor
  • -hyperthyroidism
21
Q

What is insulin-resistant diabetes?

A

Type II hypersensitivity

  • -target–Insulin receptor
  • -Hyperglycemia/ketoacidosis
22
Q

What is Pernicious anemia?

A

Type II hypersensitivity

  • -Target–intrinsic factor of gastric parietal cells–decreased absorption of B12
  • -abnormal erythropoiesis/anemia
23
Q

What is Systemic lupus erythematosus?

A

Type III hypersensitivity
Antigen–Nuclear–circulating or planted in kidney
Nephritis/skin lesions/arthritis

24
Q

What is post streptococcal glomerulonephritis?

A

Type III hypersensitivity
Antigen–streptococcal cell wall–may be planted in glomerular basement membrane
Nephritis

25
Q

What is Polyarteritis nodosa?

A

Type III hypersensitivity
Antigen– Hep B virus
Systemic vasculitis

26
Q

What is Reactive arthritis?

A

Type III hyper sensitivity
Antigen– Bacterial
Acute arthritis

27
Q

What is Serum sickness?

A

Type III hypersensitivity
Antigen–various proteins
Arthritis/vasculitis/nephritis

28
Q

What is Arthus reaction?

A

Type III hypersensitivity
Antigen–Various proteins
Cutaneous vasculitis

29
Q

What is Rheumatoid arthritis?

A

Type IV hypersensitivity
Inflammation mediated by TH17
Chronic arthritis with inflammation destruction of articular cartilage

30
Q

What is Multiple sclerosis?

A

Type IV hypersensitivity
Protein antigens in myelin
Inflammation mediated by TH1 and TH17–myelin destruction by activated macrophages
Demyelination in CNS with perivascular inflammation and paralysis

31
Q

What is type 1 DM

A

Type IV hypersensitivity
Antigens of pancreatic islet B cells
T-cell mediated inflammation–destruction of islet cells by CTLs
Insulitis/destruction of B cells/Diabetes

32
Q

What is Inflammatory bowel disease?

A

type IV hypersensitivity
enteric bacteria
Inflammation mediated by TH1 and TH17
Chronic intestinal inflammation/obstruction

33
Q

What is Psoriasis?

A

Type IV hypersensitivity
Inflammation mediated by TH17
Destructive plaques in the skin

34
Q

What is contact sensitivity?

A

Type IV hypersensitivity
Inflammation mediated by TH1
Epidermal necrosis/dermal inflammation–causing skin rash and blisters