FA Flashcards by Rebekah Lucien

What are the primary opsonins?

Which are involved in anaphylaxis? Which helps neutrophil chemotaxis? Which causes cytolysis by MAC?

Primary opsonins: C3b (also clears immune complexes) and IgG
Anaphylaxis: C3a, C4a, C5a
Chemotaxis: C5a
MAC cytolysis: C5b-9

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What are the activators for the various complement cascades?

Classic-IgG or IgM
Alternative-microbe surface mcs
Lectin- mannose on microbe surface

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What are the complement inhibitors? What disorders occur when they are absent?

C1 esterase inhibitor and DAF

hereditary angioedema (avoid ACEi)
complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria

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What cytokines do macrophages release?

IL1: Causes fever and acute inflammation
IL6: production of acute phase proteins
IL8: chemotaxis for neutrophils
IL12: Th1 differentiation
TNF-alpha: septic shock, cachexia in malignancy

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What do IL2 and 3 do? What secretes them?

All T cells, 2: stimulates growth of T cells and Nk cells

3: stimulates bone marrow, like GM-CSF

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What cytokines do helper T cells release?

IL 4: class switching to IgE and IgG, Th2 differentiation
IL 5: class switching to IgA, B cell differentiation

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What secretes interferon-gamma? What does it do?

Th1; Stimulates macrophages after stimulation from macrophages’ IL-12

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Which cytokines are anti-inflammatory?

TGF-beta and IL-10

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What defect do patients with CGD have? How do they present?

NADPH oxidase, cant make own reactive oxygen species to kill bacteria.
Granulomas, severe bacterial and fungal infections
-Present with catalase positive organisms: Listeria, Aspergillus, Candida, E coli, S aureus, and Serratia

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What are the lab findings in CGD?

Abnormal dihydrohodamine test and negative nitroblue tetrazolium dye reduction

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How does C3 and C5-9 deficiencies present?

C3: severe recurrent pyogenic sinus and respiratory tract infections, inc susceptibility to type III reactions
C5-9: terminal complement def, inc susceptibility to Neisseria bacteremia

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What surface proteins do T cells have?

All: TCR, CD28, CD3-signal transduction
Helper: CD4, CD40L
Cytotoxic: CD8
Regulatory: CD25, CD4

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What proteins do b cells uniquely have?

CD19-21

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How do superantigens like Strep pyogenes and Staph aureus cause cytokine release?

Cross link the beta region of TCR to MHC II

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How do endotoxins cause problems?

Gram neg bacteria: Stimulate macrophages directly by binding to TLR4/CD14

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Which diseases are prevented with passive immunity after exposure?

Tetanus, botulism, HBV, Varicella, Rabies

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What is releases immediately in type 1 hypersensitivity? Where does it work? What is released later on?

Histamine- postcapillary venules

Arachidonic acid metabolites: leukotrienes

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What are the three mechanisms of type II hypersensitivity?

Opsonization, complement and Fc receptor inflammation, antibody mediated cell destruction leading to MAC

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How is type 3 hypersensitivity started?

Antigen-antibody complexes activate complement, causing neutrophils to release lysosomal enzymes

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What causes serum sickness? How does it present?

Drugs

-Fever, urticaria, arthralgia, proteinuria, lymphadenopathy 5-10 days after exposure

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Which disorders are associated with type 1?

Atopic and anaphylaxis

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Type 2 disorders?

Acute hemolytic transfusion, GBS, ITP

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Type 3 disorders?

Arthus reaction, vasculitis: SLE, Polyarteritis nodosa, PSGN

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Type 4 disorders?

Contact dermatitis, GVHD, MS, PPD

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Which population is at risk for anaphylaxis reaction?

IgA deficiency

What type of reactionis febrile nonhemolytic transfusion reaction? How does it present?

Type II; fever, headaches, chills, flushing

How does acute hemolytic transfusion reaction present?

Fever, hypotension, tachycardia, flank pain, either hemoglobinuria (intravascular hemolysis) or jaundice (extravascular)

Which immunodeficiency presents with recurrent bacterial and enteroviral, Giardia infections after 6 months, typically a boy?

X-linked, Bruton agammamglobinemia, BTK defect, no B cell maturation.

What are the findings of Bruton agammaglobinemia?

Absent B cells in peripheral blood, low Ig, absent/scant LN

What are the findings of selective IgA deficiency? Are the majority sick?

Low IgA, normal IgG, M. Most asymptomatic. Can see airway and GI infections

How does common variable immunodeficiency present?

Acquired in 20s-30s, defect in B cell differentiation, low plasma cells and immunoglobulins, inc risk of autoimmune disease

How does DiGeorge present immunologically?

Dysmorphic facies, Tetany (hypoCa2+), recurrent viral/fungal infections, Cardiac: ToF, TA

What is the deletion in DiGeorge? What embryologically fails to develop?

22q11, 3rd and 4th pharyngeal pouch -> absent thymus and parathyroids

How does IL12 receptor deficiency present? What's the cause?

Disseminated mycobacterial and fungal infections; dec Th1 response causing low IFN gamma

What disease presents with coarse facies, cold staph abscesses, retained primary teeth, high IgE and derm problems (eczema)

Autosomal dominant hyper IgE syndrome

What is the defect in hyper IgE ? Labs?

Def of Th17 due to STAT3 mutation; high IgE and low IFN-gamma

What does an absent cutaneous response to Candida represent?

T cell dysfunction

What immunodeficiency presents with FTT, chronic diarrhea, mucocutaneous Candida, recurrent viral, fungal, protozoal infections? How is it treated?

Severe combined immunodeficieny; bone marrow transplant

What are the possible defects in SCID? What are the findings?

IL-2R gamma chain or adenosine deaminase deficiency; absence of thymic shadow and germinal shadows

What immunodeficiency presents with cerebellar defects, spider angioma, and IgA def

Ataxia-telangiectasia

What is the defect and findings of Ataxia-telangiectasia?

ATM (failure to repair double strand breaks); high AFP, low IgA, G, and E

How does Hyper IgM syndrome present?

severe pyogenic infections early in life, opportunistic infections (Pneumocystis, Crypto, CMV)

What is the defect in Hyper IgM? Findings?

CD40L, class switching defect. Low IgG, IgA, IgE

What disease presents with thrombocytopenic purpura, eczema, recurrent infections? Defect?

Wiskott-Aldrich; mutation in WAS gene (T cell cant reorganize actin cytoskeleton)

Which immunodeficiency presents with absent pus formation, delayed umbilical cord separation, recurrent bacterial skin infections

Leukocyte adhesion def integrin (CD18), impaired migration

What disease presents with recurrent staph and strep pyogenic infections, oculocutaneous albinism, peripheral neuropathy? Defect?

Chediak-Higashi syndrome

What is the defect in Chediak-Higashi syndrome? Findings?

Lysosomal trafficking regulator gene (LYST), microtubule dysfunction; Giant granules in granulocytes; pancytopenia

What type of transplant rejection presents with widespread thrombosis of graft vessels? How is it treated?

Hyperacute (within minutes); Caused by pre-existing antibodies (type II)

What type presents with graft vessel vasculitis with dense interstitial lymphocytic infiltrate? Treatment?

Acute rejection (weeks to months), CD8 T cells against donor MHCs and antibodies. Treat with immunosuppresion

What type presents with proliferation of vascular smooth muscle proliferation and parenchymal fibrosis, arteriosclerosis

Chronic (months to years): Recipient T cells present donor peptides to CD4 T cells

How does Graft v Host present?

Maculopapular rash, jaundice, diarrhea, HSM

What is the cause of GVHD?

Immunocompetent T cells against host. Can be helpful in leukemia

Which drugs are calcineurin inhibitors? What do they bind to?

Cyclosporine (cyclophilin) and Tacrolimus (FKBP)

What is the effect of cyclosporine and tacrolimus? What's toxicity?

Block T cell activation and preventing IL 2 transcription; Nephrotoxicity

What does Sirolimus do? Toxicity?

mTOR inhibitor, binds FKBP; prevents IL2 response from T cell activation -not nephrotoxic, anemia, thrombocytopenia, leukopenia

What does daclizumab, basiliximab block?

IL2R

What is azathioprine?

Antimetabolite precursor of 6 mercaptopurine, blocks nucleotide syntheis

Toxicity? What med increases risk

Leukopenia, anemia, thrombocytopenia, increased by allopurinol since 6MP is degraded by xanthine oxidase

What do glucocorticoids inhibit?

NF kappa B, suppresses B and T cell function

What disease does an AIRE mutation cause? How does it present?

Autoimmune polyendocrine syndrome. hypoparathyroidism, adrenal failure, chronic candida

What causes Autoimmune lymphoproliferative syndrome?

Defect in Fas (most common), FASL, or caspases leading to loss of peripheral tolerance. PResents as cytopenias, HSM, and LAD

Regulatory T cells have which 3 receptors?

CD4, CD25 (IL-2R) and FoxP3

What diseases are associated with CD25 polymorphism?

MS and T1DM

What disease does FOXP3 mutation cause?

Immune dysregulation, polyendocrinopathy (thyroiditis or DM), enteropathy (diarrhea), X-linked (IPEX syndrome) in infants

How is Libman-Sacks endocarditis differentiated from other forms?

Vegitations are present on both sides

What type of hypersensitivity is SLE in general? which one is involved in anemia, thrombocytopenia, or leukopenia?

Type III (immune complexes); type II (direct antibodies)

What labs are falsely abnormal in the presence of antiphospholipids?

VDRL and RPR (must test FTAB to determine its not syphillis) and elevated PTT

Which drugs cause drug-induced lupus?

Isoniazid, hydralyzine, procainamide

What type of hypersensitivity reaction is Sjogren's?

Lymphocyte mediated, type IV

What risk factors are associated with the presence of Anti SSA and SSB?

Extraglandular manifestations, AntiSSA crossing the placenta causing neonatal lupus and heart block

Sjoren's increases the risk of which malignancy? Presentation?

B cell (marginal) lymphoma, unilateral parotid enlargement

The following markers correlate with which autoimmune diseases: anti-Histone, Anti-centromere, anti-topoisomerase I, anti U1 RNP

anti histone= drug induced lupus, anti-centromere=CREST, antitopoisomerase/Scl 70: diffuse Scleroderma, anti U1 RNP= mixed connective tissue disease

What is the marker for hematopoetic stem cells?

CD34

Which mineral is essential for collagen scar formation?

Zinc

What does FGF do?

Its important for angiogenesis and skeletal development

Which deficiencies cause delayed wound healing and why?

Zinc co-factor for collagenase, vit C (co-factor for hydroxylation of proline and lysine), copper: co-factor for lysyl oxidase which cross links lysine and hydroxylysine

FA Flashcards

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