PATHO LE2: Immuno

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PATHO LES IMMUNO
Jessica Margaux Mercado
Karteikarten von Jessica Margaux Mercado, aktualisiert more than 1 year ago
Jessica Margaux Mercado
Erstellt von Jessica Margaux Mercado vor etwa 9 Jahre
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NKT cell surface receptor CD8
Suppressor T-cells (pan T-markers, receptor/s) CD2 CD3 CD5 CD8
Helper T-cell (receptor/s) TCR CD4
TH1 (antibody, action, secretion) IgG opsonization, complement fixing IL-2, IFN-gamma
TH2 (antibody, action, secretion) IgE activate eosinophil IL-4, IL-5, IL-13
B-cell receptor (for maturation and secretion of Ig) CD40
NK cell (surface markers, receptor/s) CD16 CD56 CD8
pan B marker CD19
indicates B lymphoid lineage (marker) Surface Ig Cellular Ig
pan T-cell Ab CD2 CD3 CD5 CD7
most specific markers for T-cells CD2 CD3
TCR: recognize peptide antigens displayed by MHC alpha/beta TCR
TCR: invariant; involved in transduction CD3 Zeta chain dimer
TCR: recognize peptides, lipids, small molecules, w/o requirement for display by MHC gamma/delta TCR
promote B cell switching to plasma cells to produce IgE; further activation of more CD4+ cells to become TH2 cells (interleukin?) IL-4
most potent eosinophil activator (interleukin?) IL-5
aids in IgE production (interleukin?) IL-13
HLA-B27 Ankylosing spondylitis, psoriasis, inflammatory bowel disease
HLA-B8 Grave's, SLE
HLA-DR2 MS, SLE, Goodpasture's
HLA-DR3 DM, SLE
HLA-DR4 RA, DM Type I, Pehmphigus vulgaris
HLA-DR5 Pernicious anemia, Hashimoto's thyroiditis
HLA-DQ3 Alopecia areata
graft rejection: preformed antibody causing immediate vascular injury via ADCC Hyperacute Rejection
graft rejection: days, months, years later; via T-cell mediated cytotoxic damage Acute Cellular Rejection
kidney rejection: early: subendothelial inflamm & hypertrophy of endothelium; intermediate: moderate intimal prolif. w/ more significant wall inflamm; severe: significant fibrinoid necrosis & intimal prolif.; via cell-mediated & humoral immunity Acute Vascular Rejection
kidney rejection: intimal fibrosis w/ vascular thickening; mononuclear infiltrates w/ prominent plasma cells; hyalinized stroma; both T-cell and humoral mechanisms Chronic Rejection
cell necrosis of skin & GIT, cholestasis (phase of GVHD) Acute Phase
over 100 days post-transplant dermal fibrosis, desquamative esophagitis, portal tract fibrosis, cholestasis (phase of GVHD) Chronic Phase
organs prominently involved in GVHD liver, intestine, skin
marked cholestasis, yellow-green bile pigment; jaundice, fine scaling rash, vacuolization of epidermal cells Graft-vs-Host Disease
most common cause for liver transplantation in children Biliary Atresia
Histocompatability required in what organs? kidney, bone marrow
w/in 2mos; mixed inflammatory portal and central vein infiltrates (liver rejection) Acute Rejection
continued inflammation, portal fibrosis, arteriolar thickening, bile ductular necrosis (liver rejection) Chronic Rejection
heart transplant: lymphocytic infiltrates, possible myocardial fiber necrosis Acute Cellular Rejection
heart transplant: immunoglobulin deposition in small arteries = vasculitis Acute Vascular Rejection
rim pattern fluorescence anti-dsDNA, anti-nuclear envelope proteins; SLE
speckled pattern fluorescence extractable nuclear antigen; Mixed Connective Tissue Disease
nucleolar pattern fluorescence anti-nucleolar RNA; progressive Systemic Sclerosis
centromere pattern fluorescence anti-centromeric protein; CREST
SLE antibodies anti-dsDNA anti-Smith anti-cardiolipin
Drug-induced SLE antibody Anti-histone
Sjörgen's Syndrome antbody Anti-SS-A, Anti-SS-B
PSS Antibody Anti-DNA-topoisomerase I (Scl-70)
Polymyositis Antibody Anti-histidyl-tRNA synthetase (Jo-1)
MCTD antibody Anti-ribonucleoprotein
malar skin rash, discoid skin rash, photodermatitis, serositis, glomerulonephritis, cytopenias, arthralgias, myalgias, vasculitis, dec C1q, thrombosis SLE
genes associated w/ SLE HLA DR-2, DR-3
drugs that can produce SLE Procainamide, Hydralazine, Isoniazid, D-Penicillamine
skin plaques w/ varying degrees of edema, erythema, scaliness, follicular plugging, skin atrophy surrounded by elevated erythematous border; usually affect face and scalp; no renal involvement Discoid Lupus Erythematosus
SLE: what part of the skin is involved? Dermal-epidermal junction
skin: chronic inflammatory infiltrates, vasculitis, vacuolization and dissolution of epidermis, purpura w/ RBC extravasation into upper dermis SLE
SLE: immune complexes seen in dermal epidermal junction? IgG, C3
vascular thrombosis in dermis; in vitro: interfere w/ coagulation; in vivo: hypercoagulable, recurrent arterial & venous thrombosis, fetal loss Antiphospholipid Syndrome
APS: autoantibodies directed against? anionic phospholipids; cardiolipin
glomerulus: thickened capillary loops, "wire loop" capillaries, surrounding renal tubules are unremarkable Lupus Nephritis
high titers of antibodies to ribonucleoprotein particle-containing U1 ribonucleoprotein MCTD
modest renal involvement, pulmonary hypertension, interstitial lung disease; overlap of SLE, scleroderma, polymyositis MCTD
calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangectiasis; associated malabsorption -> riboflavin deficiency -> cheilosis Limited Scleroderma/ CREST Syndrome
renal arterial intimal thickening and proliferation; hyperplastic arteriosclerosis -> malignant HPN w/ arterial fibrinoid necrosis, thrombosis & renal infarction Diffuse Scleroderma
diffuse alveolar fibrosis = honeycomb fibrosis; morphea Diffuse Scleroderma
symmetrical inflammation of skeletal muscle w/ weakness; skin rash; heliotropic rash Polymyositis-Dermatomyositis
fibrosis of lacrimal and salivary gland, xerostomia Sjörgen's Syndrome
lacrimal and salivary gland inflammation of any cause Mikulicz's Syndrome
failure of B cell precursors to develop into mature B cells X-linked Agammaglobulinemia/ Congenital Agammaglobulinemia/ Bruton's Disease
inheritance of Bruton's Disease X-linked recessive
Bruton's Disease: genetic defect involved? affect production of? Xq21,22 Bruton tyrosine kinase, btk
absent B-cells, intact T-cell immunity; multiple infections w/ bacterial organisms; not apparent until about 6mos Bruton's Disease
normal numbers of circulating B cells; hyperplastic germinal centers; NO plasma cells Common Variable Immunodeficiency
impaired secretion of on or more Ig (usually IgG, IgA); selective abnormality of T cell activation (dec synthesis of IL-2, 4, 5) Common Variable Immunodeficiency
dec. in CD4 cells or increase in CD8 cells; presence of T and B cell autoantibodies Common Variable Immunodeficiency
genetic defect in DiGeorge Syndrome specific deletion of 22q
field defect of 3rd and 4th pharyngeal pouch development in utero DiGeorge Syndrome
total absence of thymic tissue; hypocalcemia, seizures Complete DiGeorge Syndrome
slight dec. in peripheral T cells; inc. infections but w/ less frequency and severity; hypocalcemia, seizures Partial DiGeorge Syndrome
very little serum IgG, virtually no IgM or IgA SCID
defect in X-linked SCID: mutation? mutation in Xq = defective gamma chain of IL-2 receptor
Autosomal Recessive SCID: defect? lack of adenosine deaminase (ADA) = impaired purine metabolism
infants develop: candidiasis, persistent diarrhea, severe respi tract infection w/ Pnuemocystis carinii, Pseudomonas, failure to thrive after 3mos SCID
inheritance of Wiskott-Aldrich Syndrome? X-linked recessive
thrombocytopenia, eczema, dec IgM, inc IgA & igE; T-cells: cytoskeletal disorganization, loss of microvilli, little CD43 Wiskott-Aldrich Syndrome
genetic defect in Ataxia Telangiectasis? 11q; predisposes to chromosome breakage & rearrangement on chromosomes 7 & 14
inheritance of Ataxia Telangiectasia Autosomal recessive
progressive cerebellar ataxia, mucocutaneous telangiectasia, recurrent respi tract infection Ataxia Telangiectasia
lack circulating and secretory IgA; impaired survival of IgA producing plasma cells Selective IgA deficiency
develop anti-IgA antibodies of the IgE type Selective IgA deficiency
giant cytoplasmic granules; leukopenia (what disorder? inheritance?) Chediak-Higashi Syndrome autosomal recessive
defect in Chronic Granulomatous Disease? lack of NADPH oxidase
macrophage-rich chronic inflammatory reaction; susceptibility to catalase-positive agents Chronic Granulomatous Disease
profound suppression of T-cell mediated immunity, opportunistic infection, secondary neoplasm, neurologic disease AIDS
major targets of HIV infection T helper cells, Macrophages, Monocytes, Dendritic cells
lymphopenia, dec T cell function in vivo & vitro, polyclonal B cell activation, altered monocyte/macrophage function AIDS
Viremia; sore throat, myalgias, fever, rash, weight loss, fatigue, cervical adenopathy, diarrhea, vomiting; resolves spontaneously (what phase?) Acute Retroviral Syndrome
persistent lypmhadenopathy w/ fever, rash, fatigue; period of clinical latency (what phase?) Middle Chronic Phase
fever of more than 1 month duration, fatigue, weight loss, diarrhea, serious opportunistic infection, secondary neoplasms, clinical neurologic disease (what phase?) Final/Full Blown Progression to AIDS
CDC HIV: Category 1 greater than or equal to 500cell/uL
CDC HIV: Category 2 200-499 cell/uL
CDC HIV: Category 3 < 200 cells/uL
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