Term
| Source of salmonella antigen variation? |
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Definition
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Term
| Source of Neisseria gonorrhea antigen variation? |
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Definition
|
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Term
Hypersensitivity type?
Eczema |
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Definition
|
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Term
Hypersensitivity type?
Hemolytic anemia |
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Definition
|
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Term
Hypersensitivity type?
Pernicious anemia |
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Definition
|
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Term
Hypersensitivity type?
Idiopathic thrombocytopenic purpura |
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Definition
|
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Term
Hypersensitivity type?
Erythroblastosis fetalis |
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Definition
|
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Term
Hypersensitivity type?
Rheumatic fever |
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Definition
|
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Term
Hypersensitivity type?
Grave's Disease |
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Definition
|
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Term
Hypersensitivity type?
SLE |
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Definition
|
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Term
Hypersensitivity type?
Polyarteritis Nodosa |
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Definition
Type III
(assoc. with HBV) |
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Term
Hypersensitivity type?
Poststreptococcal glomerulonephritis |
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Definition
|
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Term
Hypersensitivity type?
Arthus reaction |
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Definition
Type III (local)
ex. swelling, necrosis, following tetanus vaccine |
|
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Term
Hypersensitivity type?
Serum sickness |
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Definition
| 5-10 days after drug exposure - fever, uticarias, arthralgias, proteinuria, lymphadenopathy |
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Term
Hypersensitivity type?
Hypersensitivity pneumonitis |
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Definition
Type III
"farmer's lung" - any exposure to organic dusts |
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Term
Hypersensitivity type?
Type 1 DM |
|
Definition
|
|
Term
Hypersensitivity type?
Multiple Sclerosis |
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Definition
|
|
Term
Hypersensitivity type?
Guillain-Barré syndrome |
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Definition
Type IV
(often vaccine or Campylobacter jejuni associated) |
|
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Term
Hypersensitivity type?
Hashimoto's thyroiditis |
|
Definition
|
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Term
Hypersensitivity type?
Graft-versus-host disease |
|
Definition
|
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Term
Hypersensitivity type?
Henoch Schonlein Purpura |
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Definition
Type III
Hinges (arthritis), Stomach (abd pain), Pee (renal involvement), + purpura
IgA immune complexes |
|
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Term
Bruton's agammaglobulinemia
Defect?
Presentation?
Labs? |
|
Definition
XLR, BTK tyrosine kinase - no B cell differentiation
Recurrent bacterial infxn after 6 mo, no opsonization
Normal pro-B, no mature B, no immunoglobulins |
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Term
Hyper-IgM syndrome
Defect?
Presentation?
Labs? |
|
Definition
Defective CD40L on Th cells - no class switch
Severe pyrogenic infxns early in life
High IgM, low other Igs |
|
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Term
Selective Ig deficiency
Defect?
Presentation?
Labs? |
|
Definition
Defect in isotype switching - lack of IgA most common
Sinus and lung infections, Anaphylaxis after exposure to blood with IgA
Anti-IgA antibodies |
|
|
Term
Common Variable Immune Deficiency
Defect?
Presentation?
Labs? |
|
Definition
Defect in B-cell maturation, many causes
Can be acquired in 20s-30s. Autoimmune disease, lymphoma, sinopulmonary infections
Normal B cells, low plasma cells, low Ig |
|
|
Term
DiGeorge Syndrome
Defect?
Presentation?
Labs? |
|
Definition
22q11 deletion: failure to develop 3rd and 4th branchial pouches
CATCH-22: Cleft palate, Abnl facies, Thymic aplasia (recurrent viral/fungal infxns), Cardiac defects, Hypocalcemia (no PTH)
Low T cells, PTH, Ca, absent thymic shadow on XR |
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|
Term
IL-12 receptor deficiency
Defect?
Presentation?
Labs? |
|
Definition
No Th1 response
Disseminated mycobacteria infxns
Low IFN-gamma |
|
|
Term
Hyper-IgE syndrome
Defect?
Presentation?
Labs? |
|
Definition
"Job's Syndrome"
Th cells don't make IFN-gamma, PMNs can't respond to chemotaxis
FATED: Facies coarse, Abscesses (noninflamed staphylococcal), Teeth (retained primary), IgE high, Dematitis (eczema)
high IgE |
|
|
Term
Chronic mucocutaneous candidiasis
Defect?
Presentation? |
|
Definition
T-cell dysfunction
Candida albicans infections of skin and mucous membranes |
|
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Term
SCID
Defect?
Presentation?
Labs? |
|
Definition
Severe Combined Immunodeficiency
Several types: defective IL-2 receptor (XLR), adenosine deaminase deficiency, failure to synthesize MHC II antigens
Recurrent viral, bacterial, fungal, and protozoal infections. Bone marrow transplant.
Low IL2-receptor = low T cell activation.
High adenine = toxic to T and B cells |
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|
Term
Ataxia-Telangiectasia
Defect?
Presentation?
Labs? |
|
Definition
Defect in DNA repair enzymes
Triad: Cerebellar defects (ataxia), Spider angiomas (telangiectasia), IgA deficiency
IgA deficiency |
|
|
Term
Wiskott-Aldrich syndrome
Defect?
Presentation?
Labs? |
|
Definition
XLR, Progressive deletion of T and B cells
TIE: Thrombocytopenic purpura, Infections, Eczema
High IgE and IgA, low IgM |
|
|
Term
Leukocyte adhesion deficiency (type 1)
Defect?
Presentation?
Labs? |
|
Definition
Defect in LFA-1 integrin (CD18) on phagocytes
Recurrent bacterial infxn, absent pus formation, *delayed umbilicus separation
Neutrophilia |
|
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Term
| Chédiak-Higashi syndrome Defect? Presentation? |
|
Definition
| AR; defect in microtubles, phagocytosis Recurrent pyrogenic infections by staph, strep; partial albinism, peripheral neuropathy |
|
|
Term
Chronic Granulomatous Disease
Defect?
Presentation?
Labs? |
|
Definition
Lack NADPH oxidase; low superoxide and respiratory burst from PMNs
Increase susceptibility to catalase positive organisms (ESPANS)
*Negative nitroblue tetrazolium dye reduction test |
|
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Term
Hyperacute transplant rejection
Mech?
Findings?
Timescale? |
|
Definition
Type II, Preformed anti-donor antibodies
Occlusion of graft vessels, ischemia, necrosis
Within minutes |
|
|
Term
Acute transplant rejection
Mech?
Findings?
Timescale?
Tx? |
|
Definition
Tc cells react to foreign MHCs
Vasculitis of graft vessels with dense interstitial lymphocytic infiltrate
Weeks after transplantation
Reversible with OKT3, cyclosporine |
|
|
Term
Chronic transplant rejection
Mech?
Findings?
Timescale?
Tx? |
|
Definition
T-cell and antibody vascular damage; donor-MHC1 perceived as self-MHC1 presenting non-self antigen
Obliterative vascular fibrosis, tissue fibrosis
Months to years after
Irreversible |
|
|
Term
|
Definition
Grafted T cells attack host organs; usually after marrow or liver transplant (lymphocyte rich organs)
Maculopapular rash, jaundice, hepatosplenomegaly, diarrhea |
|
|
Term
Reactive Arthritis
aka?
Findings?
Cause? |
|
Definition
aka Reiter's syndrome
seronegative spondylarthropathy (no RF)
Triad: urethritis (or enteritis), conjunctivitis/uveitis, arthritis (can't see, can't pee, can't climb a tree). 20% progress to sacroilitis
Associated with HLA B27, recent GI infection with Campylobacter, Salmonella, Shigella, Yersina |
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Term
Hereditary Angioedema
Mech?
Findings?
Tx? |
|
Definition
AD; low C1 esterase inhibitor. C1EI inhibits formation of activated C1 and Kallikrein, both of which lead to angioedema. Kallikrein does so by way of Kininogen->Bradykinin activation, Bradykinin is degraded by ACE.
Painless, non-pitting, well-circumscribed edema. Face, neck, lips, tongue most commonly, but internal organs possible.
Avoid ACE inhibitors, ACE needed to breakdown bradykinin and prevent some edema. |
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Term
Anti-phospholipid antibody syndrome
Mech?
Labs?
Findings? |
|
Definition
Anti-phospholipid antibodies; can be seen in SLE. Will have lupus anticoagulant, prolonged PTT, false-positive VDRL test.
Venous/arterial thromboembolism, repeated miscarriages. |
|
|
Term
Lymph node follicle
What happens here? |
|
Definition
B-cell activation/proliferation
Primary follicle: dense, dormant
Secondary follicle: pale center, active |
|
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Term
What is a Howell-Jolly body?
Indicates what? |
|
Definition
Basophilic spot (one little dot) in RBCs = DNA remnants.
Normally removed in spleen, presence = surgical or functional splenectomy |
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Term
|
Definition
CILK
C5a
IL-8
LTB4 (leukotriene B4)
Kallikrein
Bacterial products are exogenous chemokines |
|
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Term
Causes of increased ESR? (4)
Decreased ESR? (3) |
|
Definition
Increased: Infection, Inflammation, Cancer, Pregnancy
Decreased: Sickle cell, Polycythemia, CHF (?mech) |
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Term
| Complement: classic pathway activated by what? |
|
Definition
IgG or IgM complexes
(GM makes CLASSIC cars) |
|
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Term
| Complement: alternative pathway activated by what? |
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Definition
| Microbe surface proteins, esp. endotoxin |
|
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Term
| Which complement protein is an opsonin? |
|
Definition
|
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Term
| Which two complement proteins cause anaphylaxis? |
|
Definition
|
|
Term
| Which complement protein causes neutrophil chemotaxis? |
|
Definition
|
|
Term
| Which complement proteins form the MAC? |
|
Definition
Membrane Attack Complex
C5b, C6, C7, C8, C9 |
|
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Term
| Which complement pathway is more important for viral neutralization? |
|
Definition
Classic - C1-C4
Alternative needs bacterial endotoxin to activate (starts at C3) |
|
|
Term
|
Definition
Hereditary angioedema.
C1 breaks down kaillikrein, a bradykinin activator.
Because ACE breaks down bradykinin, DONT GIVE ACEis to C1 esterase deficient people!
Labs: Low C4 (unopposed C1 cleavage action) |
|
|
Term
|
Definition
| Recurrent pyogenic sinus and respiratory tract infections. Increase susceptibiity to type III hypersensitivity (immune complex). |
|
|
Term
| Deficiency of C5, 6, 7, or 8? |
|
Definition
| Recurrent Neisseria bacteremia. |
|
|
Term
| Deficiency of DAF (CD55), or Protectin (CD59)? |
|
Definition
"Decay-accelerating factor"
Causes paroxysmal nocturnal hemoglobinuria. Deficiency of these anti-complement proteins OR their GPI anchor.
Chronic complement mediated lysis of RBCs (not paroxysmal, nor nocturnal!) |
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