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88 Cards in this Set

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What's found in the medulla of the thymus?
Mature T cells
Epithelial reticular cells (that express self antigens)
Hassall's corpuscles
Histo appearance of thymic cortex vs medulla?
Cortex: dense
Medulla: pale
Rectum above pectinate line drains to what nodes?
Internal iliac nodes
Anal canal below pectinate line drains to what nodes?
Superficial inguinal nodes
Stimulus for development of helper T cell into either Th1 or Th2?
IL-12 --> Th1
IL-4 --> Th2
HLA DR 7 association?
Steroid-responsive nephrotic syndrome
HLA DR 5 associations?
Pernicious anemia (B12 deficiency)
Hashimoto's thyroiditis
HLA DR 4 associations?
Rheumatoid arthritis and DM type 1
Graves' disease HLA association?
HLA B8
Hemochromatosis HLA association?
HLA A3
HLA DR2 associations?
MS, hay fever, SLE, Goodpasture's
Antigen loading location for MHC I?
RER
Antigen loading location for MHC II?
Loaded following release of invariant chain in an acidified endosome
Function of beta-2 microglobulin?
Aids in transport of MHC I and antigen to cell surface
- can also form amyloid
Cytokines that enhance natural killer cell activity?
IL-12, IFN-beta, IFN-alpha
Macrophage-lymphocyte interaction?
Activated lymphocytes release IFN-gamma
Activated macs release IL-1, TNF-alpha
--> stimulate each other
Inhibition of Th1 and Th2 cells?
Th1: inhibited by IL-10 from Th2 cells

Th2: inhibited by IFN-gamma from Th1 cells
Perforin vs granzyme?
Granzyme: serine protease, activates apoptosis inside target cell
Perforin: helps deliver content of granules into target cell
- both released from cytotoxic T cells
What is granulysin?
Antimicrobial, induces apoptosis

- released from cytotoxic T cells
4 methods of generating Ab diversity?
1. Random VJ/VDJ recombination
2. Random heavy + light chain combos
3. Somatic hypermutation
4. Addition of nucleotides during recombination (terminal deoxynucleotidyl transferase)
APC - CD4 interactions?
MHC II - CD4 (with TCR)
B7 - CD28 (costimulation)
Th2 - B cell interaction?
CD40L (Th cell) - CD40R (B cell)
Th2 releases IL 4, 5, 6, 10
Virus infected cell, Th1, and CD8 T cell interaction?
MHC I (virus infected) - CD8/TCR (CD8 T cell)
Costimulation: IL 2 (Th1) - IL 2 R (CD8)
T cell location in spleen?
Periarterial lymphatic sheath (PALS) and white pulp
B cell location in spleen?
Follicles in white pulp of spleen
Blood smear findings post-splenectomy?
- Howell-Jolly bodies (nuclear remnants in RBCs)
- Target cells
- Thrombocytosis
2 factors that are decreased with splenic dysfunction?
1. Decreased IgM (--> decreased complement activation/opsonization of encapsulated organisms)
2. Decreased tuftsin (normally increases ability of macrophages to phagocytose)
Location of APCs in spleen?
Marginal zone (outer edge)
3 diseases that involve increased follicle size in lymph nodes?
HIV, RA, SLE
Medullary cords vs sinuses?
Cords: closely packed lymphocytes and plasma cells

Sinuses: reticular cells and macrophages
Area of lymph node enlarged in extreme cellular immune response?
Paracortex (houses T cells)
What happens to thymus-independent antigens?
Can't be presented to T cells by MHC (because they lack peptide component)
--> stimulate release of IgM Abs only and do NOT result in immunologic memory
What are thymus dependent antigens?
Antigens that contain a protein component
--> Class switching and immunologic memory occur after B cell interaction with Th2 cells (CD40-40L and release of IL 4, 5, 6)
What are patients with IL-12 deficiency at risk for?
Decreased Th1 response --> disseminated mycobacterial infections
- decreased IFN-gamma levels
Patients with chronic mucocutaneous candidiasis have deficient what?
T cell dysfunction
Features of Hyper-IgE syndrome (Job's syndrome)?
"FATED":
coarse (leonine) Facies
cold (non-inflamed) staph Abscesses
retained primary Teeth
IgE
Derm problems (eczema)
+ red hair
^^ Due to failure of Th cells to produce IFN-gamma --> neutrophils can't respond to chemotactic stimuli
Defective CD40L on helper T cells causes what condition?
Hyper IgM syndrome
- inability to class switch
- severe pyogenic infections early in life
Sinus, lung infections, milk allergies, diarrhea, anaphylaxis when exposed to IgA, frequent SP/giardia infections. Disease?
Selective Ig deficiency
- IgA deficiency most common --> failure to mature into plasma cells
- decreased secretory IgA
Cause and risks of CVID?
Caused by defect in B-cell maturation.

Increased risk of autoimmune disease (ITP, AIHA), lymphoma/malignancy, sinopulmonary infections, pneumonia, GI, etc
Lab findings in CVID?
Normal number of B cells
Decreased plasma cells and immunoglobulin
Differentiation of Bruton's agammaglobulinemia vs CVID?
CVID: normal # B cells

Bruton's: decreased number of B cells (normal pro-B)
Cause and presentation of Bruton's agammaglobulinemia?
Cause: X-linked recessive defect in BTK (tyrosine kinase) --> blocks pre-B to mature B cell maturation

Present with recurrent bacterial infections after 6 months (decreased maternal IgG) due to opsonization defect
2 factors that prevent complement activation on self cells?
Decay accelerating factor (DAF) and C1 esterase inhibitor
Effect of C3a and C5a?
Anaphylaxis

(C5a: also neutrophil chemotaxis)
Deficiency of C3 causes what?
Severe, recurrent pyogenic sinus and respiratory tract infections
Increased susceptibility to type III hypersensitivity reactions
Complement factors involved in alternative pathway?
C3, B, D
Complement factors involved in classic pathway?
C1, C2, C4, C3
Passive immunity is available for how long and after what exposures?
Half life of Abs ~ 3 weeks

After exposure to:
Tetanus toxin, Botulinum toxin, HBV, Rabies
Bacteria with antigenic variation?
Salmonella (flagella variants)
Borrelia (relapsing fever)
Neisseria gonorrhoeae (pilus prot)
Bacterial superantigens link what to what?
Cross-link beta region of TCR to MHC Class II on APCs
--> uncoordinated release of IFN-gamma from Th1 cells --> release of IL-1, IL-6, and TNF-alpha from macs
Molecular pathway of endotoxin/lipopolysaccharide?
Directly stimulate macrophages by binding to endotoxin receptor CD14 (no Th cell involvement)
Cell surface proteins on B cells?
Ig, CD19, CD20, CD21, CD40, MHC II, B7
Cell surface proteins on T cells?
TCR, CD3, CD28
- CD4, CD40L
- CD8
Cell surface proteins on macrophages?
MHC II, B7, CD40, CD14, receptors for Fc and C3b
Cell surface proteins on NK cells?
Receptors for MHC I, CD16 (binds Fc), CD56 (unique for NK cells)
Alpha and beta vs gamma interferon?
Alpha and beta: inhibit viral protein synthesis

Gamma: increase MHC I and II expression and antigen presentation in all cells
IL-12?
Secreted by macrophages and B cells.

Induces differentiation of T cells into Th1 cells and activates NK cells.
Actions of TNF-alpha?
Mediates septic shock. Activates endothelium. Causes leukocyte recruitment and vascular leak.
Idiopathic thrombocytopenia purpura (ITP) hypersensitivity type?
Type II hypersensitivity
Polyarteritis nodosa hypersensitivity?
Type III hypersensitivity
Hypersensitivity pneumonitis type?
Type III hypersensitivity
Graft vs host hypersensitivity type?
Type IV hypersensitivity
Hashimoto's hypersensitivity type?
Type IV hypersensitivity
Guillain-Barre hypersensitivity type?
Type IV hypersensitivity
Type 1 DM hypersensitivity type?
Type IV hypersensitivity
Multiple sclerosis hypersensitivity type?
Type IV hypersensitivity
Ab in mixed connective tissue disease?
Anti-U1 RNP (ribonucleoprotein)
Anti-desmoglein Ab?
Pemphigus vulgaris
Abs in Hashimoto's?
Antimicrosomal, Antithyroglobulin
Ab in polymyositis, dermatomyositis?
Anti-Jo-1
IL-2 receptor antibody and use?
Daclizumab
- used to prevent acute rejection of renal transplant
Rituximab mechanism and use?
Anti-CD20
- B cell Hodgkin's lymphoma
Anti-CD3 antibody and use?
Muromonab-CD3 (OKT3)
- prevent acute transplant rejection
Uses of alpha interferon?
Hepatitis B, C
Kaposi's sarcoma
Leukemias
Malignant melanoma
2 drugs for treatment of thrombocytopenia?
Oprelvekin (IL-11) and Thrombopoietin
2 drugs for recovery of bone marrow?
Filgrastim (G-CSF)
Sargramostim (GM-CSF)
IL-2 recombinant cytokine and use?
Aldesleukin
- used for renal cell carcinoma and metastatic melanoma
Sirolimus (rapamycin) mechanism and AEs?
Inhibit mTOR --> inhibit T cell proliferation in response to IL-2

AEs: hyperlipidemia, thrombocytopenia, leukopenia
Antimetabolite precursor of 6-mercaptopurine?
Azathioprine
What do cyclosporine and tacrolimus (FK506) each bind to?
Cyclosporine: bind cyclophilins
Tacrolimus: bind FK-binding protein

Both inhibit release of IL-2 and cytokines
Toxicity of cyclosporine?
Predispose patients to viral infections and lymphoma
- nephrotoxic (preventable with mannitol diuresis)
- hirsutism
Toxicity of tacrolimus?
Nephrotoxicity
Peripheral neuropathy
HTN
Pleural effusion
Hyperglycemia
Hair loss
Role of IL-10?
Modulate inflammatory response. Inhibit actions of activated T cells and Th1. Activate Th2.

Secreted by Th2 cells and regulatory T cells.
Cause of and findings in Leukocyte adhesion deficiency (type 1)?
Defect in LFA-1 integrin (CD18) protein on phagocytes

Get recurrent bacterial infections, absent pus formation, delayed separation of umbilicus.
Recurrent pyogenic infections (staph, strep), partial albinism, peripheral neuropathy. Disease?
Chediak-Higashi syndrome.

Defect in microtubular function with decreased phagocytosis.
Thrombocytopenic purpura, Infections, Eczema. Disease and cause?
Wiskott-Aldrich syndrome.

Progressive deletion of B and T cells. X linked recessive defect.
Triad in ataxia-telangiectasia?
Cerebellar defects (ataxia)
Spider angiomas (telangiectasias)
IgA deficiency
Lab values in Wiskott-Aldrich syndrome?
High IgE, IgA
Low IgM
Normal IgG

Progressive deletion of B and T cells. X linked recessive defect.