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107 Cards in this Set
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Type 1 Hypersens:
Describe Examples Diagnostics |
Free Ag crosslinks IgE on mast cells and basophils
Triggers release of histamine-->inc'd vasc perm, tissue edema WHEAL & FLARE Anaphylaxis Takes 15 mins Test = scratch test |
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Type 2 Hypersens
Describe Examples Diagnostics |
Ab's against self-->macs will start eating away
Mac-mediated tissue damage Complement Nphils Test: Coombs Ex: Hemolytic anemia (Ab mediated, pernicious anemia, erythroblastosis fetalis, Graves' Dz, Myasthenia gravis |
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Type 3 Hypersens
Describe Examples Diagnostics |
Ab's against Ag's; Ab-Ag complex gets deposited in tissues
Mac-mediated tissue damage Ex: Serum sickness SLE RA Arthus rxn (swelling and inflammn following tetanus vaccine)--takes 5-12 hours, not 15 mins like Type 1 |
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Type 4 Hypersens
Describe Examples Diagnostics |
Sensitized T-cell causes mac activation
4th = last, thus delayed reaction Takes 24-48 hours! T lymphocytes Transplant rejections TB skin tests Touching (contact dermatitis--poison ivy, oak, nickel, belt buckle) Ex: Type 1 DM MS Guillain-Barre Hashimoto's thyroiditis Graft vs Host PPD |
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Erythroblastosis fetalis:
Pathophys Treatment Presentation of neonate |
Maternal Abs (Rh-D) to fetal RBC Ag
In Rh(-) mom (lack Rh-D) will develop Rh-D when exposed to baby with RhD (but won't attack until second pregnancy with RhD positive baby) Tx: Rhogam (Anti-RhD Immunoglobulin) at 28 weeks, any traumatic event (MCA), and within 3rd week of delivery Neonate presents with: -Hemolytic anemia due to maternal Ab -Jaundice (possible kernicterus) -Hydrops fetalis -IU death Note: mom can also be exposed to RhD during miscarriage/abortion |
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Type of hypersens rxn:
Poststreptococcal glomerulonephritis |
III
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Type of hypersens rxn:
Asthma |
I
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Type of hypersens rxn:
Rheumatic Fever |
II
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Type of hypersens rxn:
Tb skin test |
IV
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Type of hypersens rxn:
Hay fever |
I
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Type of hypersens rxn:
Polyarteritis nodosa |
III
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Type of hypersens rxn:
Serum sickness |
III
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Type of hypersens rxn:
ABO blood type incompatibility |
II
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Type of hypersens rxn:
Poison ivy |
IV
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Type of hypersens rxn:
Eczema |
I
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Type of hypersens rxn:
Contact dermatitis |
IV
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Type of hypersens rxn:
Goodpasture's Syndrome |
II (anti-Glomerular BM Ab's)
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Anti-nuclear Ab
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SLE
Nonspecific! Sjogren's RA Juvenile arthritis |
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Andi-dsDNA Ab
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SLE (specific)
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Anti-smith Ab
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SLE (specific)
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Antihistone Ab
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Drug-induced lupus
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Anti-IgG Ab
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Scleroderma (RA)
Anti-IgG = Rheumatoid Factor; note: this is IgM attacking IgG |
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Anticentromere Ab
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CREST Scleroderma
C for CREST and centromere |
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Anti-Scl-70 Ab
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Scleroderma (diffuse)
ANti-Scl-70 = anti-DNA topoisomerase I |
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Anti-DNA topoisomerase I Ab
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Diffuse scleroderma
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Antimitochondrial Ab
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Primary biliary cirrhosis
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Antigliadin Ab
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Celiac dz
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Anti-basement membrane Ab
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Goodpasture's Syndrome
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Anti-desmoglein Ab
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Pemphigus vulgaris
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Anti-microsomal Ab
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Hashimoto's thyroiditis
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Antithyroglobulin Ab
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Hashimoto's thyroiditis
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Anti-Jo-1 Ab
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Polymyositis
Dermatomyositis |
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Anti-SS-A Ab
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Sjogren's
SS-A = Anti-Ro |
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Anti-SS-B Ab
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Sjogren's
SS-B = anti-La |
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Anti-U1 RNP Ab
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Mixed connective tissue dz
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Anti-smooth muscle Ab
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Autoimmune hepatitis
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Anti-glutamate decarboxylase Ab
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Type 1 DM
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c-ANCA Ab
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Wegener's granulomatosis
Microscopic polyangitis Churg Strauss Syndrome ANCA = anti-neutrophil cytoplasmic antibody |
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p-ANCA
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Other vasculitides
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MPO-ANCA
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Pauci-immune crescentic glomerulonephritis
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Anti-TSH receptor Ab
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Graves'
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Anti-ACh receptor Ab
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Myasthenia Gravis
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What processes elevate ESR?
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Polymyalgia rheumatic
Temporal arteritis Disease activity in RA, SLE Infection, inflammation (osteomyelitis) Malignancy Never diagnostic of anything! |
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Which complement is responsible for neutrophil chemotaxis?
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C5a
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Bruton's Agammaglobulinemia:
Describe |
B for boys (X-liniked)
B cell deficiency-->defective tyrosine kinase Low levels of all Igs Recurrent Bacterial infections after 6 months (once passive immunity wears off) |
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Thymic Aplasia:
Describe |
AKA DiGeorge
Failure of 3rd, 4th pouches to develop No thymus-->No T cells No PTH-->low Ca2+-->tetany Congenital defects in heart/great vessels Recurrent viral, fungal, protozoal infections Tap cheek: (Chvostek's sign) and spasms Trousseau's sign (where tighten BP cuff and get carpal spasm) |
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22q11 deletion
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90% of DiGeorge (detect with FISH)
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Severe combined immunodeficiency:
Describe |
Defect in early stem cell diff
Gene defects result in adenosine deaminase deficiency Last defense is NKCs Presentation: 1)Severe recurrent infections -Chronic mucocutaneous candidiasis -Fatal or recurren RSV, VZV, HSV, measles, flu, parainfluenza, PCP pneumonia 2) Chronic diarrhea 3) Failure to thrive (no thymic shadow on newborn CXR) |
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No thymic shadow on newborn CXR
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DiGeorge
Severe Combined Immunodeficiency |
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Chronic mucocutaneous candidiasis:
Describe |
T cell dysfn against Candida albicans
Tx: ketoconazole |
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Which immunodeficiencies are X-linked?
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WBC
Wiskott-Aldrich Bruton's Agammaglobulinemia Chronic Granulomatous Dz (+/- doesn't have to be X-linked) Hyper-iGM syndrome (3 types)--high IgM, low Ig's: If X-linked-->No CD-ligand If AR-->no CD40 |
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Wiskott-Aldrich Immunodeficiency:
Describe |
WAITER
Wiskott Aldrich Immunodeficiency Thrombocytopenia (and purpura) Eczema Recurrent pyogenic infections No IgM against capsular polysaccharides of bacteria Low IgM, high IgA X-linked |
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Truncal Eczema
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Wiskott Aldrich (WAITER)
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Ataxia-telangectasia:
Describe |
IgA deficiency
cerebellar Ataxia Poor smooth pursuit if moving target w/eyes Telangiectasias of face Inc'd risk of lymphoma, leukemia Radiation sensitivity Age of death ~25 alpha-fetoprotein usually elevated beyond 8 months (can be helpful with diagnosis) |
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IgA deficiency:
Describe |
Most appear healthy
Sinus and lung infections 1/600 European descent have this Assocd w/atopy (eczema), asthma Possible anaphylaxis to blood transfusions and blood products!! |
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Nitroblue tetrazolium dye test
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Chronic granulomatous dz
Phagocytes will engulf dye and will not be able to oxidize (no yellow to blue oxidation; will stay yellow) |
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Chronic granulomatous disease:
Tx |
Prophylactic TMP-SMX
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Defective LYST gene
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LYST = lysosomal transport
Chediak-Higashi Syndrome |
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Job Syndrome:
Pathophys Presentation |
Hyper-IgE syndrome
Deficiency of IFN-gamma-->nphils fail to respond to C5a, LTB4 Leads to high levels of IgE and ephils Triad: Eczema Recurrent cold (staph aureus abscesses--think of biblical Job with boils)--not warm bc not generating inflammatory response Frontal bossing, deep set eyes, doughy skin 2 rows of front teeth (retain primary teeth) |
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Leukocyte adhesion deficiency syndrome:
Pathophys |
Abnormal integrins-->inability of phagocytes to exit circulation
Delayed separation of umbilicus |
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Young child presents with tetany from hypocalcemia and candidiasis from immunosuppression.
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DiGeorge
T cell deficiency |
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Young child has recurrent lung infections and granulomatous lesions.
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Chronic Granulomatous Dz
Deficiency of NADPH oxidase |
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2 year-old child presents with multiple viral and fungal infections. Found to be hypocalcemic.
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DiGeorge
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Child has immune disorder with repeated Staph abscesses. IFN-gamma fails to mobilize neutrophils.
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Job's Syndrome (Hyper IgE)
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This embryonic germ cell gives rise to the thymus.
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Endoderm
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Define syngeneic graft.
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Graft from twin or clone
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Define allograft.
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Graft from nonidentical individual of same species
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Define xenograft.
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Graft from different species
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Hyperacute transplant rejection:
What is it? When does it occur? |
TYpe II hypersens rxn (Ab mediated) due to presence of preformed antidonor Abs in transplant recipient.
Occurs w/in minutes after transplanation. Occludes graft vessels-->ischemia, necrosis |
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Acute transplant rejection:
What is it? When does it occur? |
Cell mediated due to cytotoxic T cells reacting x foreing MHCs.
Occurs weeks after transplantation. Tx w/immunosuppressant (cyclosporine and OKT3) |
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Chronic transplant rejection:
What is it? When does it occur? |
T-cell and Ab-mediated vascular damage (obliterative vascular fibrosis)
Months to years post-transplantation. irreversible. Class I-MHC non-self perceived by CTLs as class I-MHC self presenting non-self antigen. |
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Graft-vs-Host Disease:
What is it? Presentation? |
Grafted immunocompetent T cells proliferate in irradiated immunocomp'd host and reject cells w/foreign proteins-->severe organ dysfn
Syx: Maculopapular rash Jaundice HSM Diarrhea Usually in BM and liver transplant |
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Cytokine which:
Promotes B cell growth, differentiation |
IL-4,5
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Cytokine which:
Produced by Th1 cells |
IL-2, IFN-gamma
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Cytokine which:
Produced by Th2 cells |
IL-4,5,10
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Cytokine which:
Secreted by T helper cells and activates macs |
IFN-gamma
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Cytokine which:
Pyogens secreted by by monocytes and macs |
IL-1,6, TNF-alpha
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Cytokine which:
Enhances synthesis of IgE and IgG |
IL-4
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Cytokine which:
Enhances synthesis of IgA |
IL-5
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Cytokine which:
Released by virally infected cells |
IFN-alpha, beta
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Patient suffers from recurrent Neisseria infections:
Relevant complement proteins |
Can't make MAC so C5-9 deficiency
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45-year old female complains of malar rash and arthritis:
Relevant antibodies |
Anti-dsDNA, Anti-Sm
ANA too but non-specific |
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After BM transplantation, patient suffers from dermatitis, enteritis, hepatitis.
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Graft vs Host Dz
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Infant with failure to thrive
HSM Neurodegeneration |
Niemann-Pick (genetic sphingomyelinase deficiency)
Cherry red spots on macula |
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Infant with hypoglycemia
Failure to thrive Hepatomegaly |
Cori's dz (debranching enzyme deficiency)
Glycogen in the liver can't undergo glycogenolysis |
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Infant with microcephaly
Rocker-bottom feet Clenched hands Structural heart defect |
Edwards' Syndrome (Trisomy 18)
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Jaundice
RUQ pain Fever |
Charcot's triad (ascending cholangitis)
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Keratin pearls on skin biopsy
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Squamous cell carcinoma
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Large rash with bull's eye appearance
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Erythema migrans from ixodes tick bite (Lyme dz: Borrelia)
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Lucid interval after traumatic brain injury
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Epidural hematoma (middle meningeal artery rupture)
"hit with a softball, knocked out, were lucid, and passed out again" |
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Male child
Recurrent infecitons No mature B cells |
Bruton's dz (x-linked agammaglobulinemia)
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Mucosal bleeding
Prolonged bleeding time |
Glanzmann's thrombasthenia (defect in platelet agg due to lack of GpIIb/IIIa)
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Multiple colon polps
Osteomas/soft tissue tumors Impacted/supernumerary teeth |
Gardner's syndrome (subtype of FAP)
Garden of excess tissue! |
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Necrotizing vasculitis (lungs)
Necrotizing glomerulonephritis |
Wegener's (c-ANCA poz)
Goodpasture's (anti-BM ab's) |
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Neonate with arm paralysis following difficult birth
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Erb-Duchenne palsy (superior trunk--C5-C6--brachial plexus injury)-->waiter's tip
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No lactation postpartum
Absent menstruation Cold intolerance |
Sheehan's syndrome (pituitary infarct)
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Nystagmus
Intention tremor Scanning speech Bilateral internuclear ophthalmoplegia |
MS
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Oscillating slow/fast breathing
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Cheyne-Stokes respirations (central apnea in CHF or inc'd intracranial pressure)
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Painful blue fingers/toes
Hemolytic anemia |
Cold agglutinin dz (autoimmune hemolytic anemia caused by mycoplasma pneumoniae, infections mononucleosis, EBV)
IgM Ab's precipitating together-->thrombosis-->hemolytic anemia |
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Painful, pale, cold fingers/toes
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Raynaud's syndrome (vasospasm in extremities)
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Painful, raised red lesions on palms and soles
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Osler's node (infective endocarditis)
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Painless erythematous lesions on palms and soles
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Janeway lesions (infective endocarditis)
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Painless jaundice
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Cancer of pancreatic head obstructing bile duct
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Palpable purpura
Joint pain Abdominal pain (child) |
Henoch-Schonlein purpura (IgA vasculitis affecting skin and kidneys)
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Pancreatic, pituitary, parathyroid tumors
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Wermer's syndrome (MEN I)
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Polyostic fibrous dysplasia
Precocious puberty Cafe au lait spots Short stature |
McCune Albright
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Draw brachial plexus.
Label nerves, innervations, and effects of injury. |
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