Hogan- Primary immunodeficiency Diseases Flashcards by Danielle Hayes

What is the most likely failure of immunity?
What about second most likely?
Least likely?

Antibody defect (B cell)
T cell disorders
innate and complements

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Leukocyte development has passed from (blank) to bone marrow BEFORE birth

liver/spleen

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Is phagocyte functionality complete in neonates?

yes

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Is complement function available to neonates?

yes via maternal passive transfer of IgG

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(Blank) will lage until antibody production is initiated.

Antibody mediated complement fixation (self)

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Is NK function available in neonates?

yes

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When is T cell function complete?

at birth :)

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T cell maturation is required to complete B cell maturation to functional antibody function. What does this mean?

It means that T cell function is available at infancy, but B cell function lags for 6-24 months. (i.e if T cells to develop, B cell functionality will suffer)

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Autoimmune problems in patient/family members suggest some difficulty with (blank) at possibly a genetic level.

B cell maturation/or tolerance issues

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Does immunodeficiency develop before or after autoimmunity?

either

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Most immune defenses are (blank) in nature as a significant number of autoimmue issues are failure of B cell maturation/isotope switiching/ or tolerance

humoral

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What are some severe infections of the innate immunity?

lymphadenitis, osteomyelitis, pneumonia, and sepsis

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Are phagocytes nondiscriminatory? WHat does this mean

yes

they attack both gram + and gram - and yeast/fungal organisms are attacked

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When you have a catalase producing organism, what kind of disorder should you be thinking about?

CGD (neutrophil disorder)

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What are some CGD organisms?

staph aureus
pseudomona aeroginosa
aspergillus fumigatus
candida
enterbacteriiaceae
nocardia/listeria

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What are common problems in B cell disorders?

otitis media
sinusitis
pulmonary infections

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What organisms are associated with B cell disorders?

moraxella
haemophilus influenzae
strep pneumoniae

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When is getting otitis media worrisome?

after middle school

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When you get an infection from a rar or odd organisms, what kind of problem should you suspect?

T cell

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What are some of the weird organisms associated with T cell problems?

pneumocystis carinii
candida: invasive (lung/esophagus)
system viral illness (CMV etc.)
mycobacterial infections: systemic

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What are the clinical characteristics of T cell deficiency?

Family history
onset before 6 months
opportunistic infection
no lymph nodes
cutaneous lesions
increased chance of cancer
failure to thrive
GvH disease after transfusion
Severe fungal/viral infection
fatality after BCG
diarrhea
hepatosplenomegaly

What are the clinical characteristics of B cell deficiency?

Famiy history (autoimmune/immunodeficiency)
onset after 6 months
recurrent virulent bacterial infections
allergy/autoimmune disease
vaccine failure
sinopulmonary infections
failure to thrive

What are the clinical characteristics of phagocytic deficiencies?

severity range mild to severe
susceptible to low grade bacteria, fungus
severe infections (pneumonia/osteomyelitis)
skin infections/ furunculosis
lymphadenitis
abscesses
delayed separation of the umbilical cord

What complement will be deficient if you get recurrent bacterial infections?

What complement will be deficient if you have nesseria infections?

C5-C8

What disease has neutrophils that have azurophilic granules?

chediak higashi

What disease has bi-lobed nucleus in the neutrophils?

specific granule deficiency

If you have neutrophilia what should you consider?

LAD d/o or infection

If you have neutropenia what should you consider?

congenital absence, autoantibody, cyclic neutropenia

If you have small platelets, and decreased number of platelets what should you consider?

WAS | Wiskott aldrich syndrome

If you have an RBC abnormality what should you consider?

autoimmune anemia and associated G6PD deficiency

If you have lymphopenia what should you consider?

SCID in an infant

If you have a genetic deficiency somewhere in the complement cascade what will the CH50 be? how else can you get this number

zero | profound sepsis with DIC: consumptive and will take 4-6 weeks to replenish cascade

If you have a genetic deficiency somewhere in the complement cascade what wil the AH50 be?

zero | could be consumption

If you AH50 and CH50 are both zero. where could you deficiency be?

C3 or C5-9 because these are shared by both assays

What are the 5 immunology labs you want to order?

``` IgG IgA IgM IgE Albumin ```

What does albumin tell you?

it determines if IgG loss is secondary

What IgA level is considered good?

any number greater than botom of measurable assay is good despite statistical reporting.

If a child has nasal polyposis what should you look for?

CF test

Before you look for a B cell deficiency in chidren, what 2 things might you look into?

nasal polyposis | immotile cilia syndrome

What can functional responses to vaccines tell you?

can tell you what antibodies arent being produced. | I.e if you respond to protein components but not polysaccharide componenets etc.

If you have IgM, what does this tell you?

that you have titer function

If you have failure in a vaccine you can do an advance test which is what?

its a booster dose that will check for memory cel function and plasma cell production.

If you give an advanced test and al the Igs are decreased what should you be thinking?

CD3, CD4, CD8, CD19, CD56 | ie check the CDs

If CD19 (which is B cells) is low, what should you be thinking is the culprit?

XLA | if all Bs and Cs are messed up think SCID

If you are suspecting T cell disorder, what should you look into?

HIV (secondary immunodeficiency) | CD3 CD4 CD8 CD19 (B cell), CD56 (NK cells)

What are some ways you can check T cell function?

lymphocyte stimulation with mitogens specific antigen studies (PPD if BCG vaccinated) TREC analysis of thymic output FISH: 22q11 (DiGeorge)

What is the sailboat that you see on a neonatal CXR?

the thymus :)

If your suspicious of a phagocyte disorder what do you look up?

``` CD11/18 flow cytometry serial neutrophil counts G6PD MPO Bactericidal assay (function) IgE ```

When does pneumonia in childhood become a red flag?

the third !!!

If someone says they had pneumonia what do you want to ask?

was there a chest xray done

There are 14 serotype pneumococcal titers and (blank) out of 14 is considered normal