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Respiratory > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

What are the 2 types of immunodeficiency?

A

primary/congenital
secondary/acquired

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2
Q

What causes primary immunodeficiency?

A

genetic or developmental defect

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3
Q

What causes secondary immunodeficiency?

A

result of disease or therapy

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4
Q

What immune system components can be targeted in primary immunodeficiency?

A

B-cell defects (most common)
T-cell defects
B + T cell defects
phagocytic deficiencies
complement deficiencies

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5
Q

What type of vaccinations cannot be administered to primary immunodeficiency patients?

A

live vaccines

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6
Q

List 3 congenital/central immunodeficiencies

A

Bruton’s agammaglobulinaemia
DiGeorge syndrome
Severe Combined Immunodeficiency (SCID)

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7
Q

What mnemonic gives the presentation of DiGeorge syndrome?

A

CATCH-22
Cardiac malformations
Abnormal face
Thymic hypoplasia
Cleft palate
Hypocalcaemia
22q sporadic gene microdeletion

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8
Q

What are the midline defects seen in DiGeorge syndrome?

A

hypertelorism (large distance between eyes)
down-slanted eyes
cleft palate

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9
Q

Inheritance of ataxia-telangiectasia

A

autosomal recessive
chromosome 11

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10
Q

How does ataxia-telangiectasia present?

A

2nd year of life as lack of balance and slurred speech

ataxia
severe eczema
ocular telangiectasia

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11
Q

What is the inheritance pattern of Wiscott-Aldrich disease?

A

X-linked recessive
WASP gene on Xp11 chromosome (defective cytoskeleton of T cells and platelets)

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12
Q

Wiscott-Aldrich disease clinical presentation

A

thrombocytopenia
eczema
recurrent sino-pulmonary infections
HSV/CMV/PCP

small platelets
reduced T cells
increased B cells
increased IgA + E
reduced specific antibodies

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13
Q

Pathophysiology of Bruton’s X-linked agammaglobulinaemia

A

absence or deficiency of a Bruton’s tyrosine kinase
maturation arrest of pre-B cells

levels of all Ig <10% normal
small tonsils

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14
Q

What is CVID and how does it present?

A

Common variable immune deficiency

onset after 2-10y
recurrent sinopulmonary infections (H.influenzae, S.pneumoniae, S.aureus)

lymphadenopathy
splenomegaly

no specific antibody production –> no response to vaccines

anti-B cell autoantibodies

associated with other autoimmune manifestations

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15
Q

How does selective IgA deficiency present?

A

no obvious susceptibility
majority of patients clinically normal

IgA<5 = recurrent/chronic sinopulmonary + GI infections

allergy, coeliac disease, ulcerative colitis, JRA, SLE

no specific treatment, treat associated problems

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16
Q

Chediak-Higashi syndrome inheritance

A

autosomal recessive
long arm of chromosome 1 (LYST)

17
Q

Pathophysiology Chediak-Higashi syndrome

A

lysosomes fail to fuse with the phagosome

neutropenia
diminished chemotaxis
giant lysosomes

18
Q

Chediak-Higashi syndrome presentation

A

easy bruising (abnormal platelets)
pyogenic infections
oculocutaneous albinism
photophobia
enterocolitis
peripheral neuropathy

19
Q

What infections are seen in patients with leukocyte adhesion deficiency?

A

widespread pyogenic bacterial infections

20
Q

What infections are seen in patients with chronic granulomatous disease?

A

intracellular and extracellular infection
granulomas

21
Q

What infections are seen in patients with G6PD deficiency?

A

defective respiratory burst
chronic infection

22
Q

What infections are seen in patients with myeloperoxidase deficiency?

A

defective intracellular killing
chronic infection

23
Q

What infections are seen in patients with Chediak-Higashi syndrome?

A

intracellular and extracellular infection
granulomas

24
Q

What can be deficient in the classical complement pathway and what does this lead to?

A

C1, C2, C4

deficiency leads to immune-complex disease

25
Q

What can be deficient in the Mannose-binding lectin complement pathway and what does this lead to?

A

MBL
MASP1
MASP2
C2
C4

deficiency of MBL leads to bacterial infections, mainly in childhood

26
Q

What can be deficient in the alternative complement pathway and what does this lead to?

A

Factor D
Factor P

deficiency leads to infection with pyogenic bacteria and Neisseria spp. but no immune-complex disease

27
Q

C3 deficiency effects

A

infection with pyogenic bacteria and Neisseria spp.

sometimes immune-complex disease

28
Q

What can be deficient in the membrane attack complex and what does this lead to?

A

C5
C6
C7
C8
C9

infection with neisseria spp. only

29
Q

Warning signs of primary immunodeficiency in a medical history

A

> 8 ear infections/year
2 serious sinusitis/year
2 pneumonias/year
deep-seated infections or infections in unusual areas/year

recurrent deep skin/organ abscesses
need for IV Abx to clear infection
infections with unusual/opportunistic organisms
family history of PID

30
Q

Warning signs of primary immunodeficiency - physical signs

A

poor growth (failure to thrive)
absent lymph nodes/tonsils
skin lesions (telangiectasia, petechiae, lupus-like rash)
ataxia, ocular lesions
oral thrush after 1 year of age
oral ulcers

31
Q

Cellular immunity tests

A

ANC (absolute neutrophil count)
ALC (absolute lymphocyte count)
Candida skin test

32
Q

Humoral immunity tests

A

Serum IgG, A, M, E

33
Q

Phagocyte function tests

A

CBC (complete blood count)
NBT test (Nitroblue tetrazolium test )

34
Q

Complement function tests

A

total haemolytic complement assay:
- classical = CH50
- alternative = AH50

35
Q

Which immunodeficiency syndromes can a bone marrow transplant be used in treatment?

A

Hyper-IgE syndrome
SCID
Wiscott-Aldrich
Chediak-Higashi
Kostmann disease

36
Q

Bone marrow transplants from which donors produce the best outcomes?

A

HLA-identical siblings

37
Q

How can primary immunodeficiencies be grouped?

A

by what part of the immune system is affected:
- antibody deficiencies (eg. CVID)
- combined immune deficiencies (eg. SCID)
- complement deficiencies (eg. C2 Deficiency and HAE)
- phagocytic cell deficiencies (eg. CGD)

38
Q

List some immunosuppressive agents

A

environmental stress
infectious diseases (eg. HIV)
malnutrition
genetic and metabolic diseases
immunosuppressive drugs
surgery and trauma splenectomy
age extremes: prematurity + old age

39
Q

List some causes of acquired iatrogenic immunodeficiency

A

caused by therapeutic intervention:
- cytotoxic drugs
- ionising radiation
- ALG (anti-lymphocyte globulin)
- cyclosporin

Respiratory (32 decks)

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