COMPLEMENT SYSTEM & IMMUNOLOGICAL DISORDER Flashcards
A set of proteins that play a role in cytolytic destruction of cellular antigens by specific antibody ; reaction is non specific to the target cell
Complement system
Act as chemotaxin
C5a, C5b, C6, C7
Act as opsonins
C3b, C4b, C5b
Classical pathway is initiated by
IgG and IgM
Membrane attack complex
C5b6789
based on activation via classical pathway
C 1423 56789
Lysis starts when ___ attaches to host cell
C8
Activation unit of classical pathway
C4, C2, C3
Stabilizer of C3 and C5 convert are
Properdin pathway / alternative pathway
Stabilizer of C3 and C5 convert are
Properdin pathway / alternative pathway
Involved in alternative pathway
C3 56789
Activating substance in classical pathway
Immune complex
Activating substance in alternative pathway
LPS (bacterial capsule) , IgA
Activating substance in lectin pathway
Mannose group on microbial cell
Recognition unit in classical pathway
C1q, C1r, C1s
Recognition unit in alternative pathway
C3, Factor B , Factor D
Recognition unit in lectin pathway
MBP, MASP-1
C3 convertase of classical pathway
C4b2a
C3 convertase of alternative pathway
C3bBbP
C3 convertase of lectin pathway
C4b2a
C5 convertase of classical pathway
C4b2a3b
C5 convertase of alternative pathway
C3bBb3bP
C5 convertase of lectin pathway
C4b2a3b
Dissociates C1r and C1s from C1q
C1 inhibitor
Cleaves C3b and C4b
Factor I
Cofactor with I to inactive C3b ; prevents binding of B to C3b
Factor H
Acts as a cofactor with I to inactivate C4b
C4 binding protein
Prevents attachment of the C5b67 complex to cell membrane
S protein (vitronectin)
Accelerates dissociation of C3 convertase ; associated with Cromer blood group
Decay accelerating factor
Membrane attack complex inhibitor
Homologous restriction Facto (HRF)
CD59
Membrane inhibitor of reactive lysis
CD59
Associated with 035 blood group
CD59
B cell co receptor for antigen with CD19 ; most common
CR2 (CD21)
Receptor for adhesion and increased activity of phagocytic cell
CR3 (CD11b/CD18)
Complement deficient in lupus-like syndrome ; recurrent infection ; atherosclerosis
C2
Complement deficient in hereditary angioedema
C1-INH
Complement deficient in PNH
DAF and//or MIRL
Assay for complement components — amount of C’ serum that can cause hemolysis of the 5% of the standardized concentration antibody-sensitized sheep RBC
CH50 assay
Assay for complement components — magnesium chloride, ethylene glycol tetraacetic acid
AH50 assay
___% is used because this is when the change in lytic activity per unit change in complement is at maximum
50%
Reporting of complement fixation test
Highest dilution showing no hemolysis
Heightened state of immune response
Hypersensitivity
Time of immediate hypersensitivity
6-24 hours
Time of delayed hypersensitivity
> 24 hours
Immediate type of hypersensitivity reactions
Type I , II , III
IgE as immune mediator ; heterologous ; anaphylaxis, allergic reactions, allergic asthma , food allergies, urticaria ; no complement involvement
Type I
IgG and IgM immune mediators ; antibody mediated cytotoxic ; autologous or heterologous ; cell destruction ; transfusion reactions, autoimmune hemolytic anemia , HDFN, drug reactions, myasthenia gravies ; Goodpsture’s syndrome ; Grave’s disease
TYPE III
IgM or IgG immune mediators ; complex mediated ; antigen-antibody complexes activate complement proteins ; neutrophils are recruited and release lysosomal enzymes
; serum sickness, Arthus reaction, lupus erythematosus, rheumatoid arthritis, drug reactions
Type III
T cells as immune mediators ; cell mediated or delayed type ; delayed type ; antigen-sensitized Th1 cells release cytokines that recruit macrophages and induce inflammation or activate cytotoxic T cells to cause direct cell damage ;
contact dermatitis ; tuberculin and anergy skin tests, hypersensitivity pneumonitis
TYPE IV
In vivo testing for immediate hypersensitivity
Skin test
Most preferred skin tests ; used in Wheal & Flare
Percutaneous
In vitro test for total serum IgE
RIST
In vitro test for allergen specific IgE
RAST
Primary mediators of type I hypersensitivity
Histamine
Heparin
ECF-A
NCF-A
Secondary mediators of type I hypersensitivity
Prostaglandins
Leukotrienes
Platelet activating factor
IL-1, IL-3, IL-4
Test for type II hypersensitivity reaction
AHG
Antigen is ____ in type III hypersensitivity reaction
Soluble
type III hypersensitivity reaction : deposition of ag-ab complex in the ____
Tissue
Test for type III hypersensitivity reaction ;
Labelled immunoassay
Tests for type IV hypersensitivity reaction
Skin tests (patch test , tuberculin test), interferon gamma release
Presence of autoreactive or autoantibodies cells
Autoimmune disease
Ability of the immune system to accept self antigen and not initiate a response against them
Self tolerance
Central autoimmune disease
BM, thymus
Peripheral autoimmune disease
Secondary lymphoid organs
Individual viral or bacterial agents contain antigens that closely resemble self-antigen
Molecular mimicry
Polio virus VP2 resembles
Acetylcholine
Measles virus P3 resemble
Myelin basic protein
Papilloma virus VP2 resembles
Insulin receptors
Microorganism can induce a local inflammatory response that recruits leukocytes and stimulates APCs to release cytokines that activate T cells
Bystander effect
Ability to bind to bot MHC class II and TCRs regardless of the antigen specificity
Super antigens
Immune complex disease characterized by overproduction of autoantibodies
SLE
SLE manifests itself by skin lesions _______
Butterfly rash or red wolf
It is SLE when _____ is present
Anti-nuclear antibodies (ANA)
PMN leukocyte with ingested LE body often in rosette formation
Le Cell
Drugs associated to Lupus
Procainamide
Hydralazine
Chlorpromazine
Isoniazid
Quinidine
Autoantibodies that are directed against antigens in the nuclei of mammalian cells
Antinuclear antibodies
Autoantibodies involved in SLE
Anti-dsDNA and Anti-Sm
Extractable nuclear antigen (uridine-rich RNA component)
Anti-Sm
Characteristics of antigen—antigens in the chromosome centromere ; CREST syndrome
Autoantibody - anti-centromere
CREST stands for
Calcinosis
Raynaud’s phenomenon
Esophageal dysmotililty
Sclerodactyl
Telangiectasia
Principle of fluorescent antinuclear antibody test
Indirect immunofluorescence
Fluorescent pattern : uniform staining of the entire nucleus
Homogenous/diffused
Diffuse staining is throughout the nucleus, but greater intensity around the outer circle surrounding the nucleus
Peripheral/outline/rim
Fluorescent pattern : discrete, fluorescent specks throughout nucleus
Speckled granules
Fluorescent pattern : prominent staining of the nucleoli
Nuclear
Fluorescent pattern : numerous discrete speckles are seen
Centromere-fine
Used to detect antibodies to dsDNA
Immunofluorescence using Crithidia lucillae
Used to determine the immunologic specificity of a positive FANA test
Ouchterlony test
Chronic, symmetric, and erosive arthritis of the peripheral joints; Progresses to joint deformity and disability
Rheumatoid arthritis
Specific antibody marker of Rheumatoid arthritis
Anticyclic citrullinated peptide
Group of immmunoglobulins that interacts specifically with the Fc potion of IgG molecules
Rheumatoid arthritis
A systemic autoimmune rheumatic disease ; Characterized by chronic inflammation of the excorine glands, most notably the ocular and salivary glands
Sjögren’s syndrome
Rare SARD that is characterized by excessive fibrosis and vascular abnormalities that affect skin and pints and progress over time to involve internal organs, most commonly the esophagus, lower gastrointestinal tract, lungs, heart and kidneys
Scleroderma
Inflammation of blood vessels
Granulomatosis with polyangiitis
Primarily affect the upper respiratory tract, lungs, and kidneys
Granulomatosis with polyangitis
New name of Goodpasture syndrome
Anti-glomerular basement membrane syndrome
Tumor marker for hepatic and testicular cancer
AFP
Tumor marker for lung cancer
ALP
Tumor marker for breast cancer
CA 15-3
Tumor marker for ovarian cancer
CAC 125
Tumor marker for colorectal cancer
CEA
Tumor marker for prostate cancer
PSA
Tumor marker for urinary bladder cancer
Nuclear matrix protein
Tumor marker for pancreatic cancer
Amylase
Tumor marker for medullary cancer
Calcitonin
Tumor marker for hematopoietic malignancies
CD-45
Patients are greater than 60 years of age and the incidence is greater in men than women
Non Hodgkin lymphoma