What is true about chronic granulomatous disease: - all F must be 45XO - suppurative lymphadenopathy key feature - low lymph in most - low of neut - rarely < 5 y.o. ppt

Suppurative Lymphadenopathy KEY. - X linked or AR - most ppt as infant - of neut fine= function is issue (can't kill so wall off = abscess) - dx: flow cytometry to measure oxidant - dx: nitroblue tetrazolium dye - cure= transplant

In chronic granulomatous dx, all (+) BUT: - male - leukocytosis - lymphadenopathy - hepatomegaly - hypogammaglobulinemia

Hypogammaglobulinemia - 2/3 male - yes lymph + big liver - yes WBC up

Immunology Flashcards by Jessica B

What is part of adaptive versus innate immune system?

Innate: immediate rxn

epithelial barrier
Phagocyte
Complement
NK cells

Adaptive: day 1-7

Humoral= B lymphocyte + Antibody
Cell Mediated= T cells

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Most common primary immunodeficiency?

IgA deficiency

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List as many warning signs known for primary immunodeficiency:

min. 4 AOM in 1 yr
min. 2 sinus in 1 yr
min. 2 mon. of Abx
min. 2 pneumonia in 1yr
**FTT
recurrent deep abscess
persistent thrush
**Need IV Abx to clear bacterial infection
min. 2 invasive infection (including septicaemia)
**Fhx primary immune

**= most predictive

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Delayed umbilical detachment, infections recur, leukocytosis. Dx?

Leukocyte adhesion defect

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Atopic eczema. Bloody stool. Draining Ears. Dx?

Wiskott-Aldrich Syndrome

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Recurrent staph abscess, staph pneumonia, dermatitis.

Hyper-IgE syndrome

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Sinopulmonary infection + telangiectasia

Ataxia-telangiectasia

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List two dx and type of bug=

humoral
cellular/combined
complement
phagocytic

Humoral= XLA, CIVD, Hyper IgM
= encapsulated (strep, h. influ)
= recurrent sinopulmonary

Cellular/Combined= SCID, DiGeorge, Hyper IgE
= virus (CMV, EBV), protozoa/fungal, mycobacteria, toxo
= candida

Complement= early or late defect
= Neisseria + encapsulated
= meningitis, septicemia

Phagocytic= CGD, LAD, Shwachman-Diamond, Congenital neutropenia
= Staph + gram (-) bugs
= abscess, cellulitis, osteo

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Screening primary immunodeficiency BW?

CBC + diff
(low lymph= cell; low neut= phagocytic)

Humoral
#= serum immunoglobulin
\+/- IgG subclasses
f'n= vaccine response

Cellular
#= flow cytometry
f'n= T cell proliferation study
f'n= TST test, candida test

Phagocytic testing
= DHR or NBT

Complement
#= C3, C4
f'n= CH50, AH50

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recurrent meningococcemia meningitis. Likely immunodeficiency?

C5 deficiency.

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How can you tell C3 versus C5 deficiency?

C3/ C4/ C1= AI like

C5-9= Meningococcal, invasive gonococcal

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How do you screen for T cell defect.

= CBC w/ diff (lymph)
= Flow cytometry (CD…)

Function
*= T cell proliferation (response to mitogen)
*= Delayed hypersensitivity (need prior exposure; candida or TST)
= ADA or PNP level
= Thymic Bx

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Deficiency in which can lead to anaphylaxis in pt with IVIG:

IgA

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Which dx is IgE NORMAL:

a. Wiskott aldrich
b. ITP
c. IgA def
d. KD
e. Ascariasis

ITP= Normal IgE

High in Ascariasis, KD, Wiskott
Can be low in IgA def

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Eczema, low plt, recurrent pneumonia or AOM. Likely Dx.

Wiskott Aldrich

X linked

“TIE”= thrombocytopenia + immunodef + eczema

“Wiskott Whiskey”= high EthAnol (IgE and IgA) makes mentation (IgM) go down.

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3 y.o. w/ AOM or pharyngitis every 1-3 mo. Likely?

Multiple viral infection

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List any traits you know of Familial Mediterranean Fever:

< 10 y.o.
1-3 d
every 1-2 mo.
trigger: exercise, stress, infection

Arab, Armenian, Turkish, Greek, Lebanese.

Fever
+ serositis (peritonitis, pleuritic, pericarditis)
+ pain (abdo, arthritis, chest)

Tx: Colchicine.
Complication: RF

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15 y.o. M has 5 episodes of pneumonia. In different areas. Most helpful:

a. immunoglobulin w/ IgG subclasses
b. PFT
c. CT chest
d. TB skin test
e. sputum Cx

Immunoglobulin w/ IgG subclasses

Nelson:
min. 2 resp bac/1 yr
min. 1 invasive bac
unusual site (brain, liver)
unusual severity
\+/- chronic oral candidiasis, FTT, infection after live vaccine etc.

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What is true about chronic granulomatous disease:

all F must be 45XO
suppurative lymphadenopathy key feature
low lymph in most
low # of neut
rarely < 5 y.o. ppt

Suppurative Lymphadenopathy KEY.

X linked or AR
most ppt as infant
# of neut fine= function is issue (can’t kill so wall off = abscess)
dx: flow cytometry to measure oxidant
dx: nitroblue tetrazolium dye
cure= transplant

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In chronic granulomatous dx, all (+) BUT:

male
leukocytosis
lymphadenopathy
hepatomegaly
hypogammaglobulinemia

Hypogammaglobulinemia

2/3 male
yes lymph + big liver
yes WBC up

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What are the most common bugs for chronic granulomatous dx?

*S. aureus
*Serratia Marcescens.
B cepacia

Which is true of Wiskott Aldrich?

AR
Poor response to protein antigen
CA risk
low IgE
leukopenia

Cellular/Combined

Immunodeficiency + Thrombocytopenia + Eczema

X linked

Poor response to polysacc antigen

IG…..EthAnol high= Mentation Low.

Lymphopenia

Poor response to polysacc vaccine.

YES- EBV associated CA, leukaemia, lymphoma

T or F: you can get paralysis w/ administration of live polio vaccine in X linked agammaglobulinemia?

True.

*Avoid live vaccine in X-linked-agamma
No lymph
No B cell
= no antibody

Which lab abnormality is seen in Ataxia-Telangiectasia?

low IgA
low IgE
IgG up

Low IgA

A for Ataxia.

PID: extensive mucutaneous candida..

T cell issue | SCID, Digeorge, WAS, hyperIgE

PID: meningitis, septicemia

Complement issue

Skin abscess, lymphadenitis, osteo

Phagocytic | CGD, LAD, Shwachman-Diamond

Recurrent Sinopulmonary only

Humoral | XLA, CVID

Post polio paralysis. Which PID?

XLA

Post bCG or varicella vaccine dissemination. Or hypo-Ca tetany infant. Which PID category?

Cellular/Combined | SCID, DiGeorge, WAS, hyper IgE

Which PID category is linked with lots of AI conditions?

Complement and Common Variable Immunodeficiency

18 mon. with arthritis, meningitidis, CSF meningitis. Which test?

``` #: C3, C4 Function: CH50, AH50 ```

What are key Severe combined immunodeficiency (SCID) features?

Lack all Ig classes Lack T + B + NK CC: recurrent diarrhea, AOM, sepsis Lack thymic XR (XLA only other one with no lymphoid tissue) Opportunistic infection. Live vaccine= ER

How is SCID tested for on the newborn screen?

No TREC TREC clip off to make T cell in thymus. If no T cell= No TREC. Tx: transplant

4 mo. Eczema. Recurrent pneumonia. Absent Thymus on XR. Likely?

SCID

Thrombocytopenia. Normal Hgb. Bad eczema. Chronic fingernail Staph infection. What finding confirm dx? - absent radi - no LN/tonsil - Draining ear - hypoplastic patellae

Draining ear= recurrent AOM common in Wiskott-Aldrich Syn Immune deficient + Low small Plt + Eczema B/L no radii= TAR.

Recurrent severe staph abscess of skin + lungs. IgE++++

HyperIgE syndrome ``` Pneumatocele= AD Dermatitis= AR form ```

What is the hallmark sign of familial Mediterranean fever?

Erysipelas-like erythema (esp over ankle) 1-3 d Fever serositis arthritis or rash

What is the SLE mnemonic?

SOAP BRAIN MD Serositis (pleurites, pericarditis) Oral ulcers Arthritis Photosensitivity ``` Blood (all low) Renal (protein) ANA Immunologic (dsDNA) Neuro (psych, sz) ``` Malar rash Discoid Rash

List 4 investigations for W/U of pt with suspected immunodeficiency.

1.** CBC + Diff (low lymph= cell mediated; low neut= phagocytic) 2. **serum immunoglobulin (humoral) 3. **vaccine titre (like tetanus, diphtheria; humoral f'n) 4. **flow cytometry (cellular function) ``` Other: - Cellular f'n= **Candida test, T cell proliferation test - Phagocytic testing = DHR or NBT - Complement #= C3, C4 f'n= **CH50, AH50 ```

Name two organ systems + two organism typically affected by granulocyte defect:

1. Skin + soft tissue 2. Lungs (pneumonia) Bugs: 1. Staph aureus 2. Serratia marcescens 3. Klebsiella and other

List four findings that suggest immunodeficiency:

1 systemic (sepsis, meningitis) 2 or more serious resp (pneumonia, cellulitis) within 1 yr Unusual site w/ serious infection (liver, brain) Unusual pathogen (pneumocystis jiroveci, aspergillus, serrait marcescent) Usual childhood bug but very severe

Multiple AOM + pneumonia. IgG, IgM normal but IgA low. Warn child about?

Selective IgA Deficiency Warn about possible anaphylaxis to blood products Warn about association w/ spur like syndrome (that gluten free may or may not help w/)

T or F: IgA deficiency may remit after d/c phenytoin (dilantin).

True.

T or F: IgA may evolve into CVID?

True.

Tips for vaccine hesitate parents:

1. Vaccine safety sys rigorous 2. vaccine recommendation based on dx epidemiology and evidence of vaccine safety 3. Kids exposed to more antigen in daily routine activities compared to routine vac schedule.

3 indications for conjugated quadrivalent vaccine for meningococcus?

Medical RF: 1. Asplenia or functional asplenia (i.e. sickle cell) 2. Properdin, factor D or complement deficiency 3. HIV Exposure RF: 1. Close contact to invasive meningococcal dx. 2. Travellers to endemic area (sub-saharan africa, Hajj) 3. Lab who work w/ meningococcus. 4. Military personnel in close quarters.

Child with ataxia. Most appropriate screening test for immunodeficiency?

Alpha fetoprotein level. | = Ataxia-telangiectasia

T or F: you should delay live vaccine until T cell competence established.

True.

What are the two primary immunodeficiencies that have absent lymphoid tissue (no tonsil, no lymph node)

XLA | SCID