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Allergy & immunology > Immunology > Flashcards

Immunology Flashcards

1
Q

What is the triad for ataxia telangiectasia

A
  1. Telangiactasia - not present at birth
  2. progressive ataxia - wheelchair bound as teen
  3. B and T cell def
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2
Q

What are Dx criteria for AT

A

low Ig A and IgG2
elevated AFP
Def = sequecing the ATM gene

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3
Q

CP of familial Mediterranean fever

A

typical acute episode lasts 1-4 days;
fever and 1 or more symptoms of sterile peritonitis, manifested as abdominal pain (90%), arthritis or arthralgia (85%), or pleuritis manifested by chest pain (20%).

Other serosal tissues, such as the pericardium and tunica vaginalis testis (acute scrotum), are rarely affected.

Erysipelas-like rash, myalgia, splenomegaly, scrotal involvement in boys, neurologic involvement, Henoch-Schönlein purpura, and hypothyroidism are other, less common clinical manifestations.

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4
Q

what are the 2 types of CMPA

A

Ig E mediated:urticaria, angioedema, resp, GI features

non IgE - GI issues

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5
Q

who should not have soy formula?

A

prems

congenital hypothyroidism - ? inbiits thyroid peroxidase

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6
Q

what formula is recommended for CMPA

A

hydrolysed protein

not soy, hard to diff Ig E med vs not, non IgE medated high allergy to soy too

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7
Q

what might be the issues if child has recurrent sinopulmonary infections with encapsulated organisms?

A

antibody mediated immunity issues - they evade phagocytosis

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8
Q

what are encapsulated organisms

A 
SHINE SKis
Strep pneumo
H. Influenza
Neisseria meningitis
E. Coli

Salmonella
Klebsiella
GAS

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9
Q

FTT, diarrhea, malabsorption, and fungal infection - where is the issue

A

T cell immunodef

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10
Q

eczema, petechia, easy bruising, bleeding disorder?

A

Wiskott-Aldrich syndrome

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11
Q

what is the immunologic defect of SCID

A

NO T cell +/- B cells

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12
Q

how does agammablobulinemia present

A 
No B cells
No lymphoid tissue
get encapsulated bacteria infections
usually ok with Viruses except entero
present at 4-6 month when mat immunity i s gone
No live vaccines!
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13
Q

what are the most common B-cell diseases

A

X linked agammaglobulinemia
common variable immunodef
Hyper igM
Ig A def

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14
Q

what are features of common variable immunodef?

A

initially normal
gradual decrease in Ig
GI - malabsorption, diarrhoea, splenomegaly - Looks like IBD
ID - recurrent resp infect
AI - low plt, hemolytic anemia, neutropenia
liver dysfunction
High risk of LYMPHOMA - 400x inc

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15
Q

What are clinical features of IgA def

A

Can only Dx after 4 yr when level at adult level
recurrent sino-pulm infection
food allergies
AI disease
celiac disease
can become CVID
TRANSFUSION RISK - need IiA A poor blood bc might have some of their own

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16
Q

What are clinical features of SCID

A 
severe infection - viral, bacterial, fungal, protozoa
FTT
chronic diarrhea
Severe eczema
could present with a GVHD
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17
Q

what lab results would make you worry about SCID

A

lymphopenia

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18
Q

what are important management

A

No blood transfusion - need CMV-, irradiated

No live vaccines

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19
Q

when should you worry about a primary immunodeficiency?

A 
presence of 1-2 should raise suspicion:
>=4 new AOM per year
>= 2 sinus infec per year
>= 2 pneumonias in one year
>= 2 MONTHS on Abx with little effect
>= 2 deep seated infections
recurrent abscesses
persistent thrush
need for IV Abx to clear and infection
FTT
Family HX of PID
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20
Q

if a child has no tonsils and no LN, what should you think off?

A

agammaglobulinemia
SCID

NO lymphatic tissue anywhere

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21
Q

if someone is doing a nitroblue tetrazolium test or a neutrophil oxidative burst suppression test, what are they looking for?

A

chronic granulomatous disease

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22
Q

what test would you do to confirm a complement def?

A

CH 50 - classical pathway
AH 50- alternative
C3 and C4

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23
Q

what common PID had NO Ig?

A

XLA

24
Q

a pt with XLA is at risk of what type of infections?

A 
ENCAPSULATED!
ENTEROVIRUS
sinopulmonary
GI
sepsis
meningitis
25
Q

how do we manage a pt with XLA

A

IV IG monthly
monitor trough IgG
regular PFT
NO live vaccine

26
Q

what PID has lymphatic tissue but does not make enough IG

A

CVID

27
Q

what lab findings would you expect with CVID

A

“NORMAL” amount of B cells on flow cytometry
Low Ig G but does not work well
Low/N - Ig A and M
Low or NO Ab to vaccines

28
Q

what PID has high risk of developing autoimmune issues?

A

CVID

20-25 %

29
Q

what PID has high risk of malignancy

A

CVID
High risk for lymphoma
and gastric malignancies

30
Q

what are the most common features of SCID

A 
Infections of all types
no lymphoid tissue
no thymus
oral thrush 
chronic diarrhea = FTT
severe eczema
31
Q

if you think a patient has SCID, what on initial investigations will help confirm your DDX

A

LOW lymphocytes

may have low Ig G and or IgM

32
Q

how do we treat SCID?

A 
Abx
Iv Ig
PJP prophylaxis
avoid transfusion - can lead to GVHD - or CMV neg, irridiated
Protective isolation
NO live vaccine but killed are OK
BONE MARROW TRANSPLANT!
33
Q

what is the triad for Wiskott-Aldrich syndrome?

A

eczema
low Plt
recurrent pyogenic infections

34
Q

what combined ID can present with bloody diarrhea and petechia?

A

WAS

35
Q

what combinedID will have LOW Ig Mbut high Ig A and E

A

WAS

36
Q

if a 2 year old presents with progressive ataxia and BW suggestive of lymphoma. Dx?

A

AT

37
Q

what is the disease progression of AT

A

ataxia starts at 18 month
telangiectasia starts at 2-4 yrs
wheelchair by early teens

38
Q

what is the mgnt of AT?

A

supportive only
no role for BMT
if have humural def - can do IV Ig

39
Q

what are the clinical features of DiGeorge syndrome

A 
Cardiac defect - interrupted aortic arch
Abnormal facies 
Thymus hypoplasia
Cleft palate - bifid uvula, high arch
Hypocalcemia - parathyroid issue
40
Q

what are the MC immune issues with DiGeorge syndrome?

A

Low T cell number and function but gets better over time
NO live vaccine until T cell number normal
if need transfusion - CMV neg, irr to avoid GVHD

41
Q

what are 3 phagocytic defects?

A

CGD
hyper Ig E
LAD

42
Q

what are 2 complement defects

A

Complement deficiencies

hereditary angioedema

43
Q

if a patient presents with recurrent bacterial and fungal infections, what should you consider as Dx?

A

CGD

44
Q

what bacteria are pt with CGD particularly at risk for?

A

catalase +,PLACES for CATS

Pseudomonas, 
Listeria, 
Aspergillus, 
Candida, 
E-coli, 
S. aureus, 
Serratia
45
Q

what is the MC presentation of patient with CGD?

A
  1. Cat + bacterial infection or fungal
  2. abcesses and granulomas
  3. IBD like symptoms
46
Q

what is the defect in CGD

A

defect in NADPH oxidase which is required to kill certain bugs
65 % X linked
rest is AR

47
Q

how do we manage CGD?

A

antibacterial and antifungal prophylaxis

BMT**

48
Q

patient presents with recurrent abscesses but they don’t seem to hurt or turn red. Dx

A

Hyper IgE syndrome
Neutrophils can’t get to the site of infection
therefore have COLD abscesses

49
Q

what are CF of hyper IgE syndrome

A 
coarse facial features
delayed teeth shedding
eczema
cold boils
bone fractures/scoliosis/jt hyperlaxity
50
Q

if a baby has delayed cord separation, what investigation will help you make the Dx

A

a CBC with very HIGH Neutrophils

if Neutrophils normal - Not LAD

51
Q

patient presents with recurrent episodes of swelling of the hands and feet, especially after stress. It is non painful. investigations and Dx

A
  1. C 1 esterase inhibitor def - AD

Dx- hereditary angioedema

52
Q

how do you manage an attacke of hereditary angioedema

A

C1 esterase inhibitor infusion

Can use androgens for prophylaxis

53
Q

what primary immonodef gets fungal infections

A

T cell defects: SCID, di george, AT, WAS
+ hyper Ig E
+ CGD

54
Q

who will have no response to vaccines

A

CVID

55
Q

who should not recieve live vaccines

A

Digeorge
SCID
Agammaglobulinemia

56
Q

who will NOT benefit from a BMT

A

AT

Allergy & immunology (2 decks)

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