Allergy/Immunology-Notes Flashcards by michelle granstrom

T cell disorders tend to lead to more of ___ infections?

B cell disorders ____ infections?

T cell disorders: increased viral and fungal

B cell disorders: increased bacterial and protozoal

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What are some B cell immunodeficiencies?

X-linked Bruton agammaglobulinemia
Selective IgA deficiency
Combine Variable Immunodeficiency
X-linked hypogammaglobulinemia
Hyper-IgM syndrome
IgG subclass deficiency

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What are some important things to know about X-linked Bruton agammaglobulinemia?

decrease in all Igs across the board!
recurrent bacterial and enteroviral infections after 6mo
absent lymph nodes, tonsil

treat with immunoglobulins and antibiotics

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What are some important things to know about Selective IgA deficiency?

Normal IgG, IgM, but decreased IgA.
airway, GI infections
autoimmune diseases (Celiac)
Atopy
Anaphylaxis to blood products with IgA

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What are some important things to know about CVID?

decreased levels of all immunoglobulins, but normal B cells.
sorta a plasma cell deficiency
may see giardiasis, malabsorption

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what are some important things to know about X-linked hypogammaglobulinemia?

decreased immunoglobulins
with GH deficiency
get short stature!!!

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What’s the deal with Hyper IGM syndrome?

normal B cells
decreased IgG, IgA
lots of IgM!

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IgG subclass deficiency?

normal B cells

decreased IgG

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What are some T cell disorders?

thymic aplasia (DiGeorge syndrome)
IL-12 receptor deficiency
Hyper IgE (Job)
chronic mucocutaneous candidiasis

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What are some important features of thymic aplasia?

22q11. 3rd/4th pharyngeal pouches don’t develop
cardiac: tetrology of fallot, truncus arteriosus
abnormal facies
thymic aplasia
cleft palate
hypoparathyroidism (hypocalcemia)

FISH to detect deletions
thymic graft, maybe bone marrow transplant

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IL-12 receptor deficiency?

decreased IFNgamma (from decreased TH1 cells)
disseminated mycobacterial and fungal infections

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Hyper-IgE Job?

coarse facies
staph abscess, recurrent staph cellulitis, retained primary teeth
increased IgE, eczema
cold abscess (no heat, redness, swelling)

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Features of chronic mucocutaneous candidiasis?

absent T cell response to candida
vaginal candidiasis in children/teens
infections of skin, mucous membranes

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What are some combined B and T cell disorders?

SCID
Ataxia-telangiectasia
HyperIGM
WAS

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Important things to know about SCID?

decreased B cells and T cells
caused by adenosine deaminase deficiency
presentation: FTT, chronic diarrhea, opportunistic infections, no thymic shadow, no germinal centers or lymph nodes

treatment: bone marrow transplant

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Ataxia telangiectasia…important things to know?

ataxia
telangiectasias
IgA deficiency
increased AFP
decreased IgA, IgG, IgE, lymphocytes

treatment: bone marrow transplant

Hyper IgM syndrome?

severe pyogenic & opportunistic infections early in life

only IgM is high, everything else is decreased

WAS?

Wiskott Aldrich Syndrome
TIE
T: thrombocytopenia, leading to purpura, hematemesis, hematochezia
I: immunodeficiency, recurrent infections
E: eczema

treat with bone marrow transplant, antibiotics, splenectomy

What are some phagocyte deficiencies?

LAD-1
Chediak-Higashi
Chronic Granulomatous Disease
MPO deficiency

Things to know about LAD-1?

recurrent bacterial, skin, mucosal infections. impaired wound healing. delayed separation of the umbilical cord
increased neutrophils in blood, but not in site of injury.

in IgE/Job, you see increased neutrophils in abscess just without heat etc.

Things to know about Chediak Higashi?

lysosomal trafficking problem
recurrent staph or strep infections
partial albinism
progressive neurodegeneration
giant granules in neutrophils

Things to know about CGD?

NADPH oxidase deficiency
increased susceptibility to catalase positive organisms (staph, klebsiella, pseudomonas, listeria, aspergillus, candida, E Coli)

tx: bone marrow transplant

MPO deficiency?

chronic fungal infections, candidiasis

especially strong presentation if there is diabetes.

Recurrent staph and strep abscesses?

Seen in Chediak Higashi (also albinism)
LAD-1 (no neutrophils in abscess)
Job (cold abscesses, primary teeth retained, eczema)
CGD (recurrent staph, klebsiella, pseudomonas)

Allergic rhinitis?

nasal blockage, rhinorrhea, sneezing, pruritis cobblestoning of posterior pharyngeal wall thick purulent colored mucus ``` tx: avoidance 2nd gen antihistamines: loratidine, certirizine, fexofenadine leukotriene inhibitor (montelukast) cromylyn corticosteroids atropine desensitization/immunotherapy ```

Allergic conjunctivitis?

can use antihistamines to treat vernal: bilateral inflammatory in preadolescent males--cobblestoning of the upper tarsal conjunctiva, may need montelukast for tx

Atopic dermatitis?

type 1 hypersensitivity

Urticaria, hives, angioedema?

``` mast cell degranulation type 1 hypersensitivity, IgE dependent if triggered by food, drugs, viruses IgE independent (complement mediated) if heat/cold, pressure, sun, opiates, antibiotics etc. ``` antihistamines, TCAs if chronic, systemic corticosteroids if super severe

Milk protein allergy?

Drug reactions happen how?

prior exposure, form haptens

Which has negative Nikolsky sign and target lesions? TEN stevens johnson?

Stevens Johnson has neg. Nikolsky and target lesion.

What's the deal with serum sickness?

Type 3 hypersensitivity urticaria, angioedema, EM, fever, arthritis non-IgE mediated

What's the deal with Reactive Arthritis?

Reiter's Syndrome Can't see, pee, or climb a tree conjunctivitis, urethritis, arthritis several weeks after chlamydia or diarrheal illness antibodies go to the joint causing inflammation

What's the deal with Lyme Arthritis?

monoarticular diffusely swollen joint with extreme pain migratory arthralgias Synovial Fluid: neg. gram stain, translucent, yellow Tx: doxycycline unless

Rheumatic Fever?

1-3 wks post strep pharyngitis Jones criteria + ASO Major criteria: polyarthritis, carditis (endo, peri, myo), subcunodules, erythema marginatum (pink rash over trunk), syndenham chorea Minor criteria: fever, arthalgia, high ESR/CRP, prolonged PR interval tx: ASA, corticosteroids, benzathine penicillin G

Juvenile Idiopathic Arthritis?

Oligoarticular:

C1 inhibitor deficiency?

hereditary angioedema | non-inflamm edema of the limbs, face, genitalia, laryngeal edema, intestines-colicky

C3 deficiency?

severe pyogenic infection | SLE, GNP

C5-C8 deficiency?

recurrent Neisseria infections