Vol. II - Immunology and Pathology Flashcards
Colon from splenic flexure to upper rectum drains tohy
Inferior mesenteric nodes
SCID causes
defective IL-2R gamma chain (x linked, most common), adenosine deaminase deficiency (AR), RAG mutation
Pathogenesis of hyperacute transplant rejection
preformed antibodies (type II hypersensitivity) –> rejection within minutes
IFN-gamma
stimulates macrophages to kill phagocytosed pathogens. Induces IgG switching.
TH2 cytokines
IL-4, 5, 6, 10, 13 –> activate eos and IgE production
Tumor markers: Calcitonin
Medullary thyroid carcinoma
Job syndrome presentation
ABCDEF: Abscesses, retained Baby teeth, Coarse facies, Derm problems, high IgE, Fractures
IL-1
Fever and acute inflammation. Activates adhesion molecule expression on endothelium.
Leukocyte adhesion deficiency (type 1)
defect in LFA-1 integrin (CD18) –> impaired migration and chemotaxis
Thymic aplasia syndromes
Di George and velocardiofacial
IL-12 receptor deficiency
Autosomal recessive causing decreased Th1 response –> decreased IFN-gamma –> mycobacterial and fungal susceptibility
Hyper IgM syndrome
Usually X linked defect of CD40L on T cells –> defective class switching –> severe pyogenic, opportunistic, and CMV infections –> no germinal centers or other Ig classes
TH17 cytokines
IL-17, 21, 22 –> induce neutrophil inflammation
Tumor markers: Chromogranin
neuroendocrine tumors
Rapamycin indication
kidney transplant rejection prophylaxis
NK cell activity is enhanced by
IL-2, IL-12, IFN alpha and beta
mechanism if fever in acute inflammation
Pyrogens –> magrophages produce IL-1 and TNF –> increased COX in anterior hypothalamus –> increased PGE2 –> increased temp set point
Wiskott-Aldrich presentation
WATER: Wiskott-Aldrich causes Thrombocytopenia, Eczema, and Recurrent pyogenic infections
Most common primary immunodeficiency
Selective IgA deficiency
Tumor markers: alkaline phosphotase
Mets to bone and liver, Paget’s disease, seminoma
testes, ovaries, and kidneys drain to
para-aortic nodes
HLA DR4 association
rheumatoid arthritis and DM1
Pathogenesis of chronic transplant rejection
CD4s respond to donor peptide –> type II and type IV reaction –> fibrosis etc, dominated by arteriosclerosis –> rejection in months to years
Tacrolimus indication
immunospression after solid organ transplant
T-cell costimulation is
B7 on DC binds CD28 on T cell
Type I hypersensitivity
anaphylactic and atopic
IgE mediated degranulation of presensitized mast cells
Treg cytokines
TGF-beta, IL-10, 35
Sirolimus (Rapamycin) MOA
mTOR inhibitor –> prevents response to IL-2
Protein product in proliferative phase of wound healing
Collagen Type III
X linked (Bruton) agammaglobulinemia
BTK defect –> no B cell maturation –> decrease in all Ig types –> recurrent bacterial infections (after 6 months b/c maternal Abs)
cervix, superior bladder, body of uterus drain to
external iliac nodes
What tissue does not undergo coagulative necrosis after infarct
brain
IL-6
causes fever and production of acute phase proteins
TH1 cytokines
IFN-gamma and IL-2 –> activate macrophages and CTLs
C5-C9 deficiency
increased Neisseria bacteremia susceptibility
role of leukotrienes in inflammation
endothelial contraction –> swelling
Chediak-Higashi presentation
PLAIN: progressive neurodegeneration, lymphohistiocytosis, Albinism, recurrent pyogenic Infections, peripheral Neuropathy
Type II hypersensitivity
Antibody mediated
Actue phase reactants
FFiSHH P in the C: Ferritin, Fibrinogen, Serum amyloid A, Hepcidin, Haptoglobin, Procalcitonin, C-reactive protein
Azathioprine MOA
precursor of 6-MP –> blocks PRPP amidotransferase –> reduced purine synthesis –> inhibits lymphocyte proliferation
fibril protein in hereditary amylodoses
mutated transthyretin (ATTR)
B cell costimulation is
CD40 binds CD40L on Th cell
Tumor markers: CA 27-29
Breas Cancer
Ataxia Telangiectasia
ATM defect –> Can’t detect DNA damage –> accumulation of mutations –> increased AFP / risk of lymphoma and decreased IgA, IgG, and IgE + lymphopenia
Chronic Granulomatous Disease presentation
increased susceptibility to catalase positives, recurrent infections and granulomas, Dx on flow or nitroblue tetrazolium
Warm autoimmune hemolytic anemia association
IgG
Paroxysmal Nocturnal Hemoglobinuria cause
PIGA defect –> loss of GPI anchors –> no DAF –> MAC mediated hemolysis –> decreased haptoglobin and dark urine
BCL-2 is overexpressed in what disease?
follicular lymphoma (t[14:18])
Alternate macrophage activation
Th2 secretion of IL-4 and IL-13 –> repair and anti inflammatory
MHC I antigen loading occurs in
rough ER
HLA DR3 association
SLE and DM1
IL-12
induces Th1 differentiation and activates NK cells