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Immunology > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

What are secondary immunodeficiency’s

A

Components immune system present + function
Acquired diseases or Rx affect immune function
Much more common than primary but present same way - Infection
- Environment
- Disease
- Iatrogenic

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2
Q

What are environmental causes

A 
Malnutrition 
- Zinc - monocyte, B and T cells, NK
- Iron - enzymes 
Trauma
Burns
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3
Q

What diseases cause secondary immunodeficiency

A 
Infection - HIV
DM
Renal failure
Asplenia 
CLL, lymphoma, myeloma
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4
Q

What are iatrogenic causes of SID

A 
Surgery
Splenectomy
Immunosuppression
Anti-rheumatic
Anti-epileptic
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5
Q

What are PID

A

Single gene defects leading to missing or abnormal functioning cells of the immune system

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6
Q

How are PID classified

A 
Ab disorder >50%
Predominantly T cell 
Severe combined immune deficiency 
Phagocyte disorder
Complement deficiency 
Absence of polymorphism of PRR 
Autoimmune / auto-inflammatory syndromes
Unclassified
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7
Q

What do Ab disorders lead too

A

Absent B cell so no Ab
Recurrent bacterial infections
Staph, strep, H influenza

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8
Q

What do T cell disorders lead too

A 
Unusual or opportunistic infection - usually viral
PJP / CMV / herpes / toxoplasmosis 
Candida / aspergillus 
Mycobacteria / listeria 
FTT
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9
Q

When do you suspect PID

A 
Severe infection requiring hospital or IV Ax
Persistent infection that doesn't clear 
- 4+ new ear in children or 2+ adult
- 2+ serious sinus in 1 year
- 2+ pneumonia 
- 2+ deep skin or organ abscess 
- Thrush or fungal 
Unusual infections - aspergillus / CMV
RECURRENT
Chronic diarrhoea / weight loss / FTT 
FH PID
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10
Q

What can you sometimes present with

A 
Malignancy
Syndromes
FTT
Enlarged liver or spleen
Vaccination problems
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11
Q

What can phagocyte defect cause

A

Neutrophil defect
Leucocyte adhesion deficiency
Deficiency of PRR
Chronic granulomatous disease

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12
Q

What does neutrophil defect cause

A

Congenital neutropenia

Very severe immune defect

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13
Q

What does leucocyte adhesion defect cause

A

Unable to phagocytose as no CD11/CD18 for neutrophils to attach
Present like congenital neutropenia but not as severe
Normal blood WCC but can’t mount a response

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14
Q

What does deficiency of PRR lead too

A

Can’t recognise foreign antigen so can’t phagocytose

Neutrophils migrate but don’t recognise

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15
Q

How is chronic granulomatous disease inherited and what does it lead too

A

X-linked / XR

Neutrophils cannot kill as no NAPDH oxidase

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16
Q

What does it cause

A

Aspiergillus pneumonia

S.aureus abscess in lungs / liver

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17
Q

What are clinical presentation of phagocyte defects

A

Pneumonia
OM
Skin / mucous membrane infection
Liver abscess

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18
Q

What organisms are involved

A

S.aureus
E.coli
Salmonella
Aspergillus / candida

19
Q

What infections do complement defects lead too

A 
S.aureus
Strep pneumonia
Meningococcal
Often fatal meningitis 
No chemotaxis / opsonisation / lysis of organisms
20
Q

What is hereditary angioedema

A

AD CI inhibitor deficiency so can’t block bradykinin

21
Q

What does it lead too

A

Recurrent painless, non-pitting, non-pruritic, non erythematous swelling
Affects subcutaneous tissues, intestines and oropharynx

22
Q

What is emergency

A

Pharyngeal obstruction

Acute abdomen pain

23
Q

How do you Dx and treat

A

Measure C4 level of complement - will be low
CI inhibitor infusion
Anabolic steroid as prophylaxis
FFP not available

24
Q

What are most common disorders of adaptive immunity

A

B cell over T cell

25
Q

What B cell issues can you get

A

Absence mature B cells due to stop in bone marrow
Defective function
Absence of Ig production
Absence of specific class of Ig
Absence of functional Ab - there but don’t function

26
Q

What is SCID and what causes

A

Severe dysfunction in T and B cells

Caused by defect in pluripotent stem cell, lymphoid stem cells or T or B cell

27
Q

How does it present

A 
Well for first few months as maternal Ab
Persistent candida
Persistent diarrhoea
FTT
Unwell after live vaccines
Oppurtunistic infections very severe - chicken pox, PJP, CMV 
Chronic bronchiolitis
Interstitial pneumonitis
Bacterial sepsis
28
Q

How do you Rx

A

Nutritional support
Prophylactic Ax, anti-fungal + anti-viral
Ig replacement therapy
Bone marrow donor = cure

29
Q

What is DiGeorge

A

22q11 deletion syndrome

Causes T cell deficiency

30
Q

How does it present

A 
Congenital cardiac
Palate defect
Characteristic facial 
Immunoeficiency due to thymus hypoplasia 
Developmental delay / LD
Hypocalcaemia
Psychaitric 
Haematological + ALL
31
Q

What is mneumonic

A 
CATCH 22 
Cardiac abnormality 
Abnormal face
Thymus hypoplasia / T cell 
Cleft palate
Hypocalcaemia due to hypoPTH
32
Q

What are immune disorders associated

A 
Recurrent RTI
Autoimmune as no T cell 
Anaemia
Thrombocytopenia
JIA
Raynaud's
Thyroid
33
Q

How do you Rx

A

Thymus transplant

34
Q

What causes invasive fungal

A 
Problem with innate or adaptive immune system
Presenting Sx of PID
Neutropenia due to leukaemia or chemo 
Immature immune in neonate
Broad spec Ax / abode surgery
35
Q

What are main pathogens

A

Cryptococcus
Candida
Aspergillus
Pneumocystitis

36
Q

What are RF for neonatal fungal

A 
Premature
LBW
PID
Catheter
RF neutropenia
Parenteral nutrition
Steroids
H2 blocker
Abdo surgery
Broad spec Ax
Hypoglycaemia
37
Q

When is cryptococcal common and what does it cause

A

CD4 deficiency - HIV / severe immunosuppressed

Cause meningitis

38
Q

When is pneumocystis common and what does it cause

A

CD4 deficiency

Pneumonia

39
Q

When is Aspergillus common and what does it cause

A

Innate neutrophil disorder

Pneumonia / invasive / asprgilloma

40
Q

When is Candida common and what does it cause

A

Systemic = phagocytic
Mucosal = T cell
Blood stream / urinary / skin

41
Q

How do you investigate PID

A

FBC - reduced WCC suggest T, reduced neutrophils suggest phagocyte
Serum IgG or IgA - B cell disorder
Measure complement activation
Immunophenotype
HIV test if relevant
Sweat test for CF
CXR / CT for bronchiectasis / scarring from infections

42
Q

How do you manage PID

A 
Ig if B cell deficient 
Gene therapy - SCID
Stem cell transplant - CGD / SCID
Thymus transplant - DiGeorge
Genetic counselling
43
Q

What should you avoid

A

Live vaccine

Blood transfusion

Immunology (7 decks)

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