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42 Cards in this Set

  • Front
  • Back
Hereditary angioedema results from:
Deficiency in C1 esterase
Recurrent pyogenic sinus and URI infections result from this deficiency:
C3
C6-C8 deficiency causes
Neisseria bacteremia
Paroxysmal Nocturnal Hemoglobinuria is caused by:
Deficiency in Decay accelerating factor DAF (inhibits C3 convertase)
Function of Interferon alpha and beta:
Upregulate ribonuclease production that targets viral RNA
Interferon gamma function:
stimulates MHC production and antigen presentation. Activates NK cells (all three types activate NK cells)
Cause of chronic granulomatous disease
Lack of NADPH oxidase prevents superoxide production, leaving phagocytes unable to kill organisms.
Job's syndrome: cause and characteristics:
TH1 cells fail to produce IFN-gamma, which leads to decreased macrophage activation
FATED:
Abnormal facies
Abscesses
Retained primary teeth
Increased IgE levels
Derm problems (eczema)
IgA function:
Prevents attachment of bacteria and viruses to mucous membranes, found in secretions
IgG
most abundant antibody in serum, seconary response to antigens, fixes complement, crosses placenta
IgE
Mediates type I hypersensitivity reactions, mediates immunity to worms and parasites.
IgM
Primary response to antigen, found on the surface of B cells, fixes complement
B cell function
Produce antibodies, activated by Th2 cells to differentiate into plasma cells (which secrete larges amounts of Ig A/G/E) Cause immediate organ rejection
CD8 T Cells
initiate apoptosis in virus infected cells. Express MHC I receptors.
CD4 T cells
Activate other components of adaptive immunity. Express MHC II receptors, activate B cells, activate CD8 t cells.
X-linked agammaglobulinemia, Bruton Disease
X-linked.
Onset around 6 mos
Virtually no mature B cells, no antibodies produced. Susceptible to encapsulated bacteria and enteroviruses.
Common variable immunodeficiency (CVID)
Hypogammaglobulinemia - usually low in IgA and IgG. Increased lymphoma and autoimmune suggest T cell pathology.
Selective IgA deficiency
Susceptible to bacterial infections of mucus membranes.
DiGeorge syndrom
Small deletion in chromosome 22 results in: CATCH22
Cardiac abnormalities
Abnormal Faces
Thymic hypoplasia
Cleft Palate
Hypocalcemia
22 deletion
Treatment: bone marrow transplant
SCID (severe combined immunodeficiency)
Insufficient B cells and T cells. Multiple illnesses early in life. Treatment - bone marrow transplant. IL-2 receptor defect.
Wiskott-Aldrich Syndrom
X linked mutation in protein that links TCR to cytoskeleton.
Recurrent pyogenic infection, eczema, Hi IgA, Lo IgM
Ataxia Telengiectasia
RARE AR disorder causing a defect in DNA repair enzymes. Low IgA. Ataxia - wheelchair bound. Telangiectasia - small dilated vessels on bulbar conjunctiva and skin.
Type I hypersensitivity reaction characterized by
IgE response direct at innocuous environmental antigens.
Atopy
Hypersensitivity affecting parts of the body not in direct contact with the allergen. examples: asthma eczema allergic rhinitis urticaria anaphylasis.
IgE levels are sensitive to:
IL-4 from Th2 cells increases
IFN gamma from Th1 cells is inhibitory.
Type II hypersensitivity
IgG or IgM is directed against antigen on self cells, or transfused blood cells. Leads to phagocytosis or complement mediated lysis.
Hemolytic disease of newborn
type of type II hypersensitivity reaction. Mom is Rh neg, baby is positive.
Goodpasture's syndrome
glomerular and lung basement membranes provoke immune response. Type 2 hypersensitivity.
Myasthenia gravis
circulating antibodies (IgG) attach to acetylcholine receptors on muscle membranes. Type 2 hypersensitivity.
Graves disease
overactive thyroid as a result of stimulating antibodies for TSH receptor. Type 2 hypersensitivity.
Type 3 hypersensitivity reactions aka
immune complex reactions
Mechanism of type 3 hypersensitivity reactions
Antibody-antigen complexes form
Complexes act on complement
Complement induces histamine release
Complexes deposit on walls of small vessels
PMNs are attracted by C3a and C5a
PMNs release oxidative enzymes to try to clear complexes
Platelets form microthrombi causing ischemia
Type 4 hypersensitivity aka
delayed hypersensitivity
Type 4 hypersensitivity mediated by ____ cells
Th2 cells and IgE
Kinds of type 4 hypersensitivity diseases
Type 1 DM, Graft vs host disease, tuberculin, granulomatous
SLE
Systemic Lupus Erythematosus - Autoimmune disease. Antibodies detected: antinuclear antibody, antibodies to dsDNA, anti-smith antibody, antihistone antibody (drug induced SLE)
Pathogenesis of autoimmunity
Suppressor T cells reduce risk of autoimmune disease.
Thymectomy increases risk.
B cells promote autoimmunity through antibody production.
T cells are required for rejection because...
Th cells release cytokines to induce activation of CD8+ cells, B cells, and macrophages.
Cyclosporin mechanism of action
Inhibits calcineurin in T cells, preventing intracellular signaling and production of IL2 needed for differentiation and activation of T cells.
Cancers linked to Epstein Barr virus
Lymphoma, burkitt's lymphoma, nasopharyngeal carcinoma.
In immunosuppressed: virus infects more B cells, for which it is mitogenic.
Cancers linked with HPV
Cervical cancer, anorectal cancer. Preference or columnar epithelial cells near squamous junctions. Inclusion of DNA into host genome.
Tumor associated transplantation antigen
Exposure to inactivated tumor leads to immunity to other active tumors of the same type.