10. Immunocompromised host Flashcards

(32 cards)

1
Q

“immunocompromise” is?

A

state in which the immune system is unable to respond appropriately and effectively to infections microorganisms

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2
Q

“immunodeficiency” is?

A

immunocompromise caused by defect in 1 or more components of immune system

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3
Q

describe the 2 types of immunodeficiency

A
1. primary - congenital
due to intrinsic gene defect (275 genes)
- missing protein 
- missing cell
- non-functional components
  1. secondary - acquired
    due to underlying disease/treatment
    - decreased production/function of immune components
    - increased loss or catabolism of immune components
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4
Q

Which 4 characteristics of infections suggest underlying immune deficiency?

A

SPUR:

Severe
Persistent (despite treatment)
Unusual (site or microogranism)
Recurrent

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5
Q

10 warning signs of PIDs in children?

A
  1. 4+ new ear infection <1yr
  2. 2+ serious sinus infections <1yr
  3. 2+ pneumonias <1yr
  4. recurrent, deep skin or organ abscesses
  5. persistent thrush in mouth or fungal infection on skin
  6. 2+ deep-seated infections inc. septicaemia
  7. 2+ months on antibiotics with little effect
  8. need for IV antibiotics to clear infections
  9. failure of infant to gain weight/grow normally
  10. family history of PID
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6
Q

10 warning signs of PID in adults?

A
  1. 2+ new ear infections <1yr
  2. 2+ new sinus infections <1yr
  3. 1 pneumoniae/yr for >1yr
  4. recurrent viral infections
  5. recurrent deep abscesses of skin or internal organs
  6. persistent thrush or fungal infection on skin or elsewhere
  7. infection with normally harmless tuberculosis-like bacteria
  8. recurrent need for IV antibiotics to clear infections
  9. chronic diarrhoea with weight loss
  10. family history of PID
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7
Q

what are the limitations of the 10 warning signs

A
  1. lack of pop-based evidence (e.g. family history, failure to thrive)
  2. PID patients with diff. defects/presentations (infections with subtle presentation, T cells/ B cells/ phagocytes/ complement)
  3. PID patients with non-infectious manifestations (autoimmunity, malignancy, inflammatory responses)
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8
Q

which malignancy is common in PIDs

A

lymphoma

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9
Q

name the 4 main types of PID in commoness order, and give an example of each

A
  1. predominantly antibody deficiencies (65%)
    - COMMON VARIABLE IMMUNODEFICIENCY (CVID)
    - X-LINKED AGAMMAGLOBULINAEMIA (BRUTON’S DISEASE)
    - SELECTIVE IgA DEFICIENCY (usually asymptomatic)
  2. combined B and T cell
    - SEVERE COMBINED IMMUNODEFICIENCY (SCID)
  3. phagocytic defects
    - CHRONIC GRANULOMATOUS DISEASE
  4. other cellular immunodeficiencies
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10
Q

what PIDs do different ages of symptom onset suggest

A
  • <6 mths = T cell or phagocyte defect
  • > 6 mths - <5 yrs = B cell/antibody or phagocyte defect
  • > 5 yrs = B cell/antibody defect, complement defect or secondary immunodeficiency
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11
Q

which microbes are commonly associated with complement deficiency

A

encapsulated bacteria, e.g. Neisseria spp, streptococci, H. influenzae

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12
Q

which infections are associated with C3 or C5-C9 deficiency?

A

C3 = pyogenic infections

C5-C9 = meningitis, sepsis and arthritis

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13
Q

which condition results from C1 inhibitor deficiency?

A

angioedema

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14
Q

which microbes are associated with phagocytic defects?

A

Bacteria: S. aureus, P. aeruginosa, non-tuberculous mycobacteria

Fungi: Candida spp., Aspergillus spp.

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15
Q

which infections are associated with phagocytic defects?

A
  • skin/mucous infections
  • deep seated infections
  • invasive fungal infections (ASPERGILLOSIS)
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16
Q

which specific infection is indicative of antibody deficiencies

A

giardia lamblia (protozoa) GI infection

17
Q

which microbes are associated with antibody deficiencies?

A

Bacteria: streptococci, staphylococci, H. influenzae, P. aeruginosa, Mycoplasma pneumoniae (upper resp. infections)

Protozoa: Giardia lamblia

18
Q

which infections are associated with antibody deficiencies

A
  • sinorespiratory infections

- GI infections

19
Q

which conditions are associated with antibody deficiencies

A
  • arthropathies
  • malignancies
  • autoimmunity
20
Q

which microbes are associated with T cell deficiencies

A

Bacteria: same as antibody deficiency + intracellular bacteria like S. typhi, L. monocytogenes, and non-tuberculous mycobacteria

All viruses

Fungi: Candida spp, Aspergillus spp, Cryptococcus neoformans, Histoplasma capsulatum

Protozoa: Pneumocystis jirovecii, Toxoplasma gondii, Cryptosporidium parvum

21
Q

which conditions are associated with T cell defects?

A
  • opportunistic infections
  • deep skin and tissue abscesses
  • failure to thrive
  • death if not treated
22
Q

what is supportive treatment for PID?

A
  1. infection prevention (prophylactic antimicrobials)
  2. treat infections promptly and aggressively (passive immunisation)
  3. nutritional support (vitamins A/D)
  4. use UV-irradiated CMVned blood products only
  5. avoid live attenuated vaccines in Ps with severe PIDs (SCID)
  6. avoid non-essential exposure to radiation
23
Q

what is the specific treatment for antibody deficiencies?

A

regular immunoglobulin replacement therapy (IVIG or SCIG)

24
Q

what is the specific treatment for SCID

A

Haematopoietic Stem Cell Therapy (90% success), but:

  • need donor match
  • must be performed within 3 1/2 mths
25
name commorbidities of PID
1. autoimmunity 2. malignancies (esp. lymphoma and leukaemia) 3. organ damage (e.g. lungs) if untreated
26
what is immunoglobulin replacement therapy and when is it used
concentrated form of Igs given either IV every 3 wks or Sc (subcutaneous) in young patients used to treat CVID, XLA and hyper-IgM syndrome
27
what are the main causes of secondary immune deficiencies?
decreased production of immune components 1. malnutrition - main cause 2. infection (HIV) 3. liver diseases (as liver products acute phase proteins, etc.) 4. lymphoproliferative diseases - i.e. cancer and chemotherapy (causes neutropenia) 5. splenectomy
28
name possible causes for splenectomy/hyposplenism
- infarction (eg sickle cell anaemia) - trauma - autoimmune haemolytic disease - infiltration (tumour) - coeliac disease - congenital
29
why is the spleen so important
largest lymphoid organ 1. only organ to fight blood-borne pathogens, inc. encapsulated bacteria 2. antibody production: - acute response: IgM production (pentameric - 5 fold activation of complement) - long term protection: IgG production (best opsonin) 3. splenic macrophages: - removal of opsonised microbes - removal of immune complexes
30
what are the complications of splenectomy
1. increased susceptibility to encapsulated bacteria: H. influenzae, Strep. pneumoniae, N. meningitidis 2. OPSI (overwhelming post-splenectomy infection): sepsis and meningitis
31
describe the management of asplenic patients
1. penicillin prophylaxis (life-long) 2. immunisation against encapsulated bacteria 3. medic alert bracelet
32
why does haematological malignancy increase infection susceptibility?
1. chemotherapy-induced neutropenia 2. chemotherapy-induced damage to mucosal barriers 3. vascular catheters (e.g. Hickman line)