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CSP 3 > Immune deficiency > Flashcards

Immune deficiency Flashcards

1
Q

Define immunodeficiency

A

State of suboptimal resistance to infectious disease

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2
Q

2 ways in which immunodeficiency can be classified?

A
  • Primary and Secondary

- By which part of the immune system has failed

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3
Q

What are the 2 types of primary immunodeficiency

A

Genetically mutation disease

Idiopathic diseases

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4
Q

Give 3 genetically mediated diseases that is a primary cause of immune deficiency

A
  • Severe combined immune deficiencies
  • X-linked agammaglobulaemia
  • Di George syndrome
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5
Q

Give an infection that is a secondary cause of immune deficiency

A
  • AIDS (sx to HIV)
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6
Q

Which malignancies commonly cause immune deficiencies

A
  • Haematological malignancies such as lymphoma, myeloma, leukemia
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7
Q

What metabolic disease notably is a secondary cause of immunodeficiency

A

Diabetes

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8
Q

What kinds of truama commonly lead to immunodeficiency

A

Burns

Penetrating injuries

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9
Q

What treatments can cause immunodeficiency

A

Glucocorticoids
Immunosuppressive agents
Cytotoxic drugs
Irradiation

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10
Q

Are primary or secondary immunodeficiencies commoner

A

Secondary

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11
Q

Which infections can lead to immunodeficiency via B-lymphocyte and antibody deficiency?

A
  • Bacterial infections with common bacteria (pneumococcus, haemophilus, etc.)
  • Viruses, especially enterovirus
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12
Q

What does agammaglobulinaemia mean

A

No antibodies

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13
Q

What gene is defective leading to agammaglobulaemia

A

Tyrosine kinase gene specific to B lymphocytes

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14
Q

How to patients present with x linked agammaglobulaemia

A

Present when aged 6 months- year with chest infections, sinusitis, otitis media etc.

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15
Q

What happens if x linked agamma globulaemia is left untreated

A
  • Inexorably worse chest infections and develop bronchiectasis and will die in childhood and adulthood
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16
Q

What antibody replacement in indicated in agammaglobulaemia

A

IgG

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17
Q

Which age group and sex get common variable immune deficiency

A

Any age

Either sex

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18
Q

What is the state of B lymphocytes and antibodies in common variable immune deficiency

A

B-lymphocytes may be normal or low

Antibodies are low but almost never absent

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19
Q

Which condition has impared test response to immunisation

A

Common variable immune deficiency

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20
Q

What are some common associations with common variable immune deficiency

A 
Autoimmunity 
Granulomatous disease (lung disease, liver)
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21
Q

How is common variable immune deficieny disease treated

A

Antibody replacement

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22
Q

Which antibody is usually deficient in isolated antibody class deficiencies

A

IgA

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23
Q

Rituximab is an antibody against…. which specifically depletes …..

A
  • CD20

- Depletes B lymphocytes

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24
Q

Name 2 primary causes of T lymphocyte deficiencies

A
  • Di George Syndrome

- Nucleoside phosphorylase and Adenine Deaminase deficiency

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25
Q

Which chromosome is deleted in Di George syndrome

A

Chromosome 22

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26
Q

Which organs are absent in Di George syndrome

A

Thymus and parathyroids

27
Q

What is the underlying problem in nucleoside phosphorylase and adenine deaminase deficiency

A

Enzyme specific to T lymphocytes

28
Q

What are the main targets of HIV

A

CD4+ cells

29
Q

What is the diagnostic test for HIV

A

Antibodies to HIV, p24 viral antigen, HIV RNA

30
Q

Do glucocorticoids affect B cells or T cells

31
Q

Common presentation of neutropenia

A

Frequently starts as oral soreness, gingivitis, severe pharyngitis
Upper and lower resp tract infections
Soft tissues infections and abscesses
Septicaemia

32
Q

What are the cellular changes in severe combined immuno-deficiency

A

Only RBC and plaltelets in the cells, all immune cells are absent

33
Q

What is deficient in chronic granulomatous disease

A

Neutrophils

34
Q

When does chronic granulomatous disease present

A

In childhood usually before 5 year old

35
Q

How does chronic granulomatous disease

A
  • Pneumonia
  • Infective arthritis
  • Osteomyelitis
  • Abscesses in organ and tissue
36
Q

What test is used to diagnose chronic granulomatous disease

A

Nitroblue tetrazolium test OR

Dihydrorhoadmine test

37
Q

Treatment for neutrophil function defects (3)

A
  • Antibiotics
  • Interferons
  • Bone marrows transplant
38
Q

What is leukocyte adhesion deficiency

A

Autosomal recessive disease- deficiency of CD18.

39
Q

What is the normal function of CD18

A

Molecule associated with CD11 to form beta 2 integrin, a neutrophil adhesion molecule. Without CD18/CD11 complex, neutrophils cannot pass through the post capillary venule walls in inflammation

40
Q

How do you test for leukocyte adhesion deficiency

A

CD11/18 expression can be measured

41
Q

How do you treat leukocyte adhesion deficiency

A

Abx, bone marrow/ stem cell transplant

42
Q

Presentation of leukocyte adhesion deficiency

A
  • Omphalatis
  • Pneumonia
  • Gingivitis
  • Peritonitis
43
Q

Name some diabetic predispositions to infectious diseases

A
  • UTI
  • Foot infections
  • Superficial fungal infections of skin
  • Disseminated candidiasis
  • Rhino-pulmonary mucormycosis
44
Q

4 possible mechanisms of how diabetes predisposes a patient to infectious disease

A
  • Neutrophil function impaired
  • Microcirculation imparired
  • Body fluids more nutritious for bacteria due to glucose conc
45
Q

Which pathway is involved in angioedema

A

Bradykinin inflammatory cascade pathway

46
Q

What deficiency leads to angioedema

A

C1 esterase inhibitor

47
Q

What is type 1 hereditary angioedema

A

Absence of C1 esterase inhibitor

48
Q

What is type 2 hereditary angioedema

A

Malfunction of C1 esterase inhibitor

49
Q

What is type 3 hereditary angioedema

A

Mutation of clotting factor 12 (as this disinhibits bradykinin patheway)

50
Q

How do you treat angioedema if it’s hereditary

A
  • Anabolic steroids to increase secretion of C1 esterase inhibitor
  • Tranexamic acid reduces trigger events in tissues
  • Icatibant inhibits bradykinin pathway
51
Q

In which blood disease is the spleen progressively infarcted

A

Sickle cell disease

52
Q

In which malabsorbative disease is hyposplenism common

53
Q

Why may the spleen be removed

A
  • Hereditary spherocytosis
  • Lymphoma
  • Hypersplenism
  • Idiopathic thrombocytopenia
  • Trauma
54
Q

Treatment of hyposplenism/ absent spleen

A
  • Immunise pt with pneumococacal vaccine and meningococcal vaccine
  • Prophylactic abx- penicillinV/ macrolide
55
Q

Which disorders can cause defects of teh anatomical innate immune system

A
  • Cystic fibrosis
  • Bronchiectasis
  • Urinary outflow obstruction
  • Defects in cilia
  • Burns/ wounds
  • Poor phlebotomy/ catherisation technique
  • Indwelling lines
56
Q

What is reticular dysgenesis

A
  • Only red cells and platelets in teh blood

- Only lilve a few days without dying

57
Q

What is Hyper IgM

A
  • Inherited defect causing loss of signal from activated CD4 T helper cell to APC
  • B lymphocytes don’t go under class switching etc.
58
Q

What mutation causes hyper IgM syndrome

A
  • Mutation of CD40 on T lymphocyte is the most common cause

- Rarely, lack of CD40

59
Q

What mutation Hyper IgE

A
  • Very high IgE
  • Eosinophilia
  • Facial and skeletal abnormalites
60
Q

What is Wiskott- Aldrich syndrome

A
  • X-linked
  • Defective cytoskeletal protein found in haemopoetic cells
  • Thrombocytopenia gives bleeding and affects B&T lymphocytes
61
Q

What is Chediak-Higashi syndrome

A

Mostly affects phagocytes
Neutropenia
Giant inclusion bodies

62
Q

What is the inheritance of ataxia telangectasia

A

Autosomal recessive

63
Q

Symptoms of ataxia talangectasia

A
  • Autosomal recessive defect in ATM gene
  • Ataxia
  • Telangectasia

CSP 3 (9 decks)

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