Immunology, Autoimmunity and Related Disorders Flashcards
Hypersensitivity can be defined as?
A normal but exaggerated or uncontrolled immune response to an antigen that can produce inflammation, cell destruction, tissue injury.
What are the 4 types of hypersensitivity?
- Type I - Anapylactic
- Type II - Cytotoxic
- Type III - Immune Complex
- Type IV - T cell dependent
An allergy refers to?
What condigtion it is related and which Ig is it mediated by?
- An altered reaction to external substances
- Related to atopy, that refers to immediate hypersensitivity mediated by IgE antibodies
Histamine is released by?
Mast cells or basophils
An allergic reactio to plasma products containing IgG may occur in individuals that are?
IgA - deficient
Ultarcartarial reations are?
- Skin rashes
- hives
- swelling from mosquito bite
- animal hair
- allergy testing
- dermatitis
- exzema
What are the two types of Anaphylaxis?
- Systemic - throat closes, brochioles swell, difficulty swallowing, difficulty breathing, shock, drop in blood pressure, fainting, loss of consiousness, immediate coughing, sneezing, cramps, vomiting, diarrhea, cramps, pain, dizziness, death if untreated.
- Local - visible rash, hives, swelling, redness, warmth, pain, itching.
A Type I allergic reaction can be mediated by what cells?ex: allergic contact dermatitis
T - lymphocytes (regulatory)
______ can be attracted to the area of activity by chemotactic factors and may release _____ mediators that ____ the effect of ______ mediators
Eosinophils
secondary
limit
primary
Hypersensitivity Type II - cytotoxic reactions are a consequence of?
IgG or IgM bindind to the surface of cells
What is the test for Type II?
DAT
What are the three mechanisms of Type II ?
What are the examples of each mechanism and how they work.
-
Antibody dependent, complement mediated cytotoxic reactions
- Ex - HTR / HDN
-
Antibody dependent, cell mediated cytotoxicity
- Goodpastures syndrome. IgG autoAbs bind glycoprotein in the basement mebrane of glomeruli and the lungs, activates complement and triggers inflammatory resonse.
-
Antireceptor antibodies
- Abs bind to cells and affect their function. Stimulate target organ function wihtout causing organ damage in some cases Wegner’s granulomatosis may lead to tissue damage.
Type III immune complex reactions are caused by?
Deposistion of immune complexes in blood vessel walls and tissues
What is an Arthus Reaction?
Repeated exposure leads to sensitization producing an insoluble antigen-Ab complex.
What are 2 common skin conditions of Type III reactions?
- Allergic Vasculitis
- Erythema nodosum
A Type III pulmonary reaction?
- Hypersensitivity pneumonitis (farmers lung - reaction to thermophilic actinomycetes found in moldy hay)
Bathtub refinisher’s lung, Epoxy resin lung, and plastic worker’s lung can be cause by what?
- Chemicals
- tolulene diisocyanate
- phthalic anhydride
- timetallic anhydride
What assays are commonly used for Type III reactions?
- Latex aggutination
- Nephelometry
- Chemiluminescence
What are Type IV reactions caused by?
Immune activities that differ fom Abs mediated immunity.
Cell mediated immunity is moderated by the link between what 2 cells?
Between T-lymphcytes and phagocytic cells
Lymphocytes (T cells) don’t recognise antigens of a microrganism or other living cells by are immunologically active via varios types of direct cell to cell contact and by the soluble factors.
True / False
True
Type IV delayed-type hypersensitivity (DTH) involves T cells that do what?
- Antigen sensitized T cells that stay phagocytized in a marcrophage and are previously encountered by activated T-lymphocytes for a second time.
- T cells respond directly or by the release of lymphokines exhibiting contact dermatitis and infection allergies
Monocloncal gammopathies are classified by?
Production of monoclonal immunoglobulin (Ab)
MAbs are involved with the supression of?
Uninvolved immunoglobulins and dysfunctional T cell responses.
MAbs result from the single clone of a lymphiod (B cell) plasma cells producing higher levels of a single class/ type of immunogloulin called?
- Monoclonal protien
- paraprotein
- M Protein
- All of the above
All of the above
Monocloncal gammophathies can be benign or malignant?
True / False
True
What is the most common plasma cell disorder?
How does age affect this disorder?
Premaligant precursor of myeloma, Monoclonal Gammopathy of Undetermined Significance (MGUS)
Incidence increases with age
What is Multiple Myeloma?
- Plasma cell neoplasm characterized by the accumulation of malignant plasma cells within the BM micronvironment.
- Monocloncal protein in the blood or urine
- Assocated organ dysfuction
In multiple myeloma the percentage of plasma cells in the BM can rise to?
90%
What factors may be a cause of multiple myeloma?
What environment stimilants may be a cause?
- Radiation or viral causes
What environment stimilants may be a cause of multiple myeloma?
- Asbestos
- Benzene
- industrial toxins
- All of the above
All of the above
What is the most common form of dysproteinemia and accounts for 1% of all malignant diseases and 10% of hemaologic malignancies?
Multiple Myeloma
What is the most common form of Multiple Myeloma
- IgG Myeloma (52% of patients)
- IgM Myeloma (52% of patients)
- IgA Myeloma (52% of patients)
- IgE Myeloma (52% of patients)
IgG Myeloma (52% of patients)
Most patients with MM die within?
Due to?
- 1 - 3 years
- Sepis and renal insufficiency
How does MM affect the skeleton and cause bone pain?
90% have broadly disseminated destruction of their skeleton
What is the most consitent feature of MM?
Incessant systhesis of a dysfuntional single monoclonal protein or of immunoglobulin chains of fragments with concurrent supression of the systhesis of normal fuctional Ab.
In 99% of MM patients what i found in urine, serum or both?
M component
Proteinuria is a common occurence in MM, with approaximaty what percentage of patients excreting what protein?
What percentage of patients only excrete this protein?
- 50% excreting Bence Jones Protein
- 10%
What is an improtant diagnostic marker of MM?
Bence Jones Protein
A common finding in the peripheral smear of patients with MM?
Rouleaux formation due to increases in monoclonal protein production.
A common B cell dissorder characterized by infiltration of lymphoplasmacytic cells in to the bone marrow and prescence of an IgM Monocloncal gammopathy?
Waldenstroms Primary Macroglobulinemia (WM)
The may be a genic cause for WM as 20% of patients are related?
True / False
True
WM occurs 10% as frequently as MM?
True / False
True
MM if often found in what age individuals with a survival rate of?
60 - 64 years
3 years after diagnosis
The following are initial symptoms of WM?
- Weakness
- Fatigue
- Bleeding from nose and gums
- all of the above
All of the above
40% of patients will WM will also develop enlargement of which organs?
- Hepatomegaly
- Splenomegaly
- Lymphomeglaly
Elevated IgM levels in WM affect eyesight causing?
Blurred vision
Contrary to MM, bone pain in WM is almost non-exisitent?
True / False
True
What affects 90% of patients with chronic uncontrolled WM?
Congestive heart failure
Micrscopic apsirate examination in an individual with WM often reveals?
Ragged cytoplasm and stain positive with periodic Acid-Schiff.
Defined as an increase in more than one immunoglobulin and involves serveral clones of plasma cells?
Polyclonal gammopathies
Polyclonal gammopthies consist of one or more heavy chain classes and both light chain class types, as opposed to monocloncal light chain configurations?
True / False
True
Polyclonal gammopathies are often seen in?
- Chronic infections
- Chronic Liver disease (esp. chronic active hepatitis)
- Rhematoid connective tissue (autoimmune) disease
- Lymphoproliferative disorders
- All of the above
- All of the above
The classic model for autoimmune disease is?
SLE
A chronic sytemic rheumatic disorder affecting multiple systems including - joints , connective tissue, collagen vascular system?
Systemic Lupus Erythematosus (SLE)
SLE can occur at any age but is more frequent in?
10 - 15 times more freq in women than men after puberty
What is the survival rate for Lupus?
90% at 10 years after diagnosis
What is a leading cause of death in hopitalized patients suffering from progressive renal disease or CNS disease?
SLE
Immunosupression from steriod treatment can interfere wit host defence against oppoertunistic infections like?
M. Tuberculosis
H. Capsulatum
L. Monocytogenes
All of the above
All of the above
What sign/ symptom is seen in 90% of SLE patients?
- Fatigue
- Weight loss
- Joint pain / arthritis
- Butterfly rash
Joint pain / arthritis
What sign/ symptom is seen in 40% of SLE patients?
- Fatigue
- Weight loss
- Joint pain / arthritis
- Butterfly rash
Butterfly rash (across nose and cheeks)
SLE increases suceptibility to
common and oportunitstic infections
Blood tests for SLE may find (3)?
- Moderate anemia
- Lymphocytopenia
- Thrombocytopenia
Serologic manifestations of SLE will show (4)?
- High levels of Antinuclear Abs (ANAs)
- Immune complexes
- Complement level depression
- Tissue depositions of immunoglobulins and complement
What is the hallmark autoAb of SLE?
Antinuclear Antibodies
ANA antobodies include:
- DNA
Histone - Non-histone protein
- All of the above
All of the above