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93 Cards in this Set
- Front
- Back
What are the 3 main types of lymphocytes and how are they identified?
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B cells, T cells, and Natural Killer cells (NK)
they are identified by cell-surface glycoproteins specific for both cell type and stage of differentiation |
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Where do B cells originate?
Where do they differentiate? |
stem cells in the bone marrow
bone marrow and peripherally in germinal centers of lymph nodes and lymphoid follicles of the spleen |
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What is a characteristic feature of B cells?
What percent of circulating peripheral blood lymphocytes are B cells? |
the presence of surface immunoglobulin
15% |
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Where do T cells originate?
Where do they differentiate? Where can mature T cells be found? What percent of circulating peripheral blood lymphocytes are T cells? |
stem cells in the bone marrow
thymus paracortical and deep medullary areas of lymph nodes and periarteriolar sheaths of the spleen 70% |
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What are the two subclassifications of T cells and how abundant are they in circulating T cells?
What is the normal ratio of CD4+ to CD8+ T cells? What is the ratio in AIDS patients? |
CD4+ T cells (60%) and CD8+ T cells (30%)
2:1 0.5:1 or less |
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What is another name for NK cells and why?
What percentage of circulating lymphocytes are NK cells? |
large granular lymphocytes (LGLs)
large size, pale cytoplasm, and prominent granulation 15% |
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What do NK cells kill?
Specific sensitization involved? Antibody involved? ADCC? |
tumor cells, fungi, and cells altered by viral infection
NO NO antibody-dependent cell-mediated cytotoxicity (ADCC) the method in which NK cells lyse other cells |
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Macrophages are derivatives of peripheral blood ___ and are members of the ___ system of cells.
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monocytes
mononuclear phagocyte |
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What are 3 things that macrophages do?
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1) secrete cytokines, acid hydrolases, neutral proteases, and prostaglandins
2) process and present antigens to CD4+ T cells (along with human leukocyte antigen (HLA) class II antigens) 3) participate in delayed hypersensitivity reactions (directly killing tumor cells) |
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What characterizes dendritic cells? (2 things)
What is the main similarity between dendritic cells and macrophages? What is the main difference between dendritic cells and macrophages? |
dendritic cytoplasmic processes and expression of large quantities of cell surface HLA class II antigens
they are both antigen-presenting cells (APC) dendritic cells are poorly phagocytic |
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What characterizes Langerhans cells of the skin?
How are they similar to dendritic cells of lymphoid tissue? |
presence of Birbeck granules (tennis racket-shaped cytoplasmic structures)
antigen-presenting cells and express HLA class II antigens |
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Give 3 types of cells that express human leukocyte antigen (HLA) class II antigens?
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macrophages, dendritic cells, and langerhans cells
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Give 3 types of cells that secrete cytokines.
What is the role of cytokines? |
lymphocytes, monocytes-macrophages, and NK cells
act as effector molecules influencing the behavior of B, T and NK cells, monocytes, macrophages, hematopoietic cells, and others |
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This system consists of 20 plasma proteins that form a membrane attack complex to lyse target cells.
How are the plasma proteins activated? |
Complement system
2 pathways: classic pathway and alternate pathway |
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What initiates the classic pathway of complement activation?
The final lytic form of activated complement is the result of _____ |
reaction with antigen-antibody complexes
a series of enzymatic cleavages and recombinations (cleavage products cause cell lysis) |
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What initiates the alternate pathway of complement activation?
Like the classic pathway, what causes cell lysis? |
nonimmunologic stimuli such as invading microorganisms
cleavage products |
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What is the name of the genes that code for HLA antigens?
Where are they located? What is the HLA system used for? |
histocompatibility genes
short arm of chromosome 6, the major histocompatibility complex organ transplantation- HLA typing and matching of donor recipient for tissue compatibility |
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What are the class I HLA antigens?
Where are they found? Class I antigens are the main antigens involved in ____. |
HLA-A, HLA-B, and HLA-C antigens
on almost all human cells tissue graft rejection (serologic testing for HLA-A and HLA-B antigens is used to predict the likelihood of long term graft survival) |
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What are the class II HLA antigens?
Where are they found? |
HLA-D, HLA-DP, HLA-DQ, and HLA-DR
immunocompetent cells (macrophages, dendritic cells, langerhans cells, B cells, and some T cells) |
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How are HLA-D antigens identified?
How are HLA-DP, HLA-DQ, and HLA-DR antigens identified? |
mixed lymphocyte reactions
standard serologic techniques or by mixed lymphocyte reactions |
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How are HLA antigens linked with disease?
What is an example? |
there is a significant associated of certain HLA antigens with a number of specific diseases
HLA-B27 antigen is associated with 90% of cases of ankylosing spondylitis |
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What mediates adaptive immunity?
What are the drawbacks? |
B and T lymphocytes and specific antigens
not immediate; take time for optimal reactivity |
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What are some examples of innate immunity?
What is innate immunity associated with? |
phagocytosis and activation of complement
toll-like receptors |
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What are hypersensitivity reactions?
Which types require the active production of antibody by plasma cells? Which type is mediated by the interaction of T cells and macrophages? |
adverse reactions caused by immune mechanisms
Types I, II, and III Type IV |
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What is type I hypersensitivity?
Steps: 1) ____ antibody is produced by ____ and is stimulated by ____. The antibody is then bound to the ___ receptors of ___ and ___ cells. 2) On subsequent exposure, ___ reacts with the bound ____, resulting in ____ and degranulation of ____ and ____ cells. Step 2 requires ____ of antibody on the surface of the ___ cell. 3) Degranulation results in ___ release, which causes what? 4) Chemotactic substances recruit ____, resulting in ____. |
immediate or anaphylactic hypersensitivity
1) IgE; IgE B cells; antigen; Fc; basophils; mast cells 2) antigen; IgE antibody; cytolysis; basophils; mast cells; cross-linking; mast 3) histamine; increased vascular permeability 4) eosinophils; tissue and peripheral blood eosinophilia |
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What are 3 examples of type I hypersensitivity?
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allergic reactions, systemic anaphylaxis (anaphylactic shock), and angioedema
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What is type II hypersensitivity?
3 pathways? |
antibody-mediated or cytotoxic hypersensitivity
complement-fixing antibodies, antibody-dependent cell-mediated cytotoxicity (ADCC), and reaction of anti-receptor antibodies with cell-surface receptor protein |
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In Type II hypersensitivity reactions, the complement-fixing antibodies react directly with antigens that are ____.
The interaction of ____ with the cell surface results in ____ and ____. Where are the antigens involved usually located? 4 examples? |
integral components of the target cell
complement; cell lysis; destruction They are usually localized to tissue basement membranes or blood cell membranes. warm antibody autoimmune hemolytic anemia, hemolytic transfusion reactions, hemolytic disease of the newborn, and Goodpasture syndrome |
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In Type II hypersensitivity reactions, the ADCC pathway begins with the antibody reacting with ____.
The ____ portion of the antibody molecule reacts with the ____ receptors of many cytotoxic leukocytes, most importantly ____. How are the target cells killed? Is complement involved? |
the antibody reacts directly with integral surface antigens of targeted cells
Fc; Fc receptors; NK cells Fc receptor-bound cytotoxic leukocytes no |
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In Type II hypersensitivity reactions, which variant is sometimes classisfied as type V hypersensitivity?
What occurs here? What disease is this? What is the result of this reaction? |
reaction of anti-receptor antibodies with cell-surface receptor protein
the reaction of thyroid-stimulating immunoglobulin with the TSH receptor of thyroid follicular cells Graves disease antigen-antibody reaction mimics the effect of TSH on the follicular cells and results in glandular hyperplasia and hyperproduction of thyroid hormone with clinical hyperthryroidism |
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Type III hypersensitivity is also called the ____.
Steps 1) ____ produced in response to antigen, results in circulating ____. 2) What usually happens to immune complexes? How can this step go wrong? 3) The immune system complexes bind ____, which is chemotactic for neutrophils. The neutrophils release ____, resulting in ____. 4) _____ is activated with promotes coagulation, causing ____ of small vessels and activation of ____ causing vasodilation and edema. 5) Finally, ___ causes microthrombus formation and leads to the release of ____ from platelet dense granules. |
immune complex
1) exogenous antibody; antigen-antibody complexes 2) usually removed by cells of the mononuclear phagocyte system; insoluble aggregates of immune complex are deposited in vessel walls and are smaller and less easily removed by phagocytes 3) complement; lysosomal enzymes; tissue damage 4) Hageman factor (XII); thrombosis; kinin 5) platelet aggregation; vasoactive amines |
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5 examples of the anti-receptor antibody reaction with cell-surface receptor proteins (Type II hypersensitivity)?
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serum sickness, systemic lupus erythematosus, arthus reaction, polyarteritis nodosa, and immune complex-mediated glomerular diseases
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Type IV hypersensitivity is also called ___.
What are the two pathways? |
cell-mediated hypersensitivity
delayed hypersensitivity and cytotoxic T lymphocyte-mediated cytotoxicity |
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In Type IV hypersensitivity, delayed hypersensitivity begins when _____ reacts with the antigen, which was presented by ____. It also reacts with ____ on the macrophages, cuasing stimulation of ____.
What happens on subsequent contact with antigen? What do the secretions stimulate? 2 examples? |
T-cell receptor of CD4+ lymphocytes; macrophages; HLA class II antigens; antigen-specific CD4+ memory T cells
CD4+ memory T cells proliferate and secrete cytokines phagocytic activity of macrophages tuberculin reaction and contact dermatitis |
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In type IV hypersensitivity, what is cytotoxic T lymphocyte-mediated cytotoxicity?
What cells are usually targeted? How are they recognized? Target cell ____ recognized as self-antigens. |
direct CD8+ T cell-mediated killing of target cells.
tumor cells or virus infected cells the specific target cell antigen is recognized by the T-cell receptor of CD8+ lymphocytes HLA class I antigens |
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what mediates hyperacute transplant rejection?
when does rejection occur? what kind of rejection is present? |
antibody-mediated
within minutes of transplant Arthur reaction marked by acute inflammation, fibrinoid necrosis, and extensive thrombosis |
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what mediates acute transplant rejection?
when does rejection occur? what kind of rejection is present? |
t-cell mediated
days or weeks after transplant infiltration of lymphocytes and macrophages |
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what mediates chronic transplant rejection?
when does rejection occur? what kind of rejection is present? |
antibody-mediated
months to years after transplant vascular damage |
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X-linked agammaglobulinemia of Bruton?
Absence of plasma cells in tissues results in ____. Absent ____ in lymphoid tissue. Effects? |
occurs in male infants, is characterized by failure of antibody synthesis due to a mutation in the B cell tyrosine kinase (Btk) gene (cannot turn pre-B cells into B cells)
absence of serum immunoglobulin germinal centers recurrent bacterial infections (steptococci, staphylococci, pneumococci) |
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What is the most common inherited B cell defect?
What causes this? effects? |
isolated IgA deficiency
inability of IgA B cells to mature to plasma cells usually asymptomatic but may have occasional anaphylactic reactions or infections |
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What is common variable immunodeficiency?
Results in ____ Manifest clinically by ___ |
group of disorders caused by failure of terminal B-cell maturation
hypogammaglobulinemia recurrent bacterial infections |
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DiGeorge syndrome?
How does this occur? Results in ___ Mainfest clinically by ___ |
congenital T-cell deficiency
hypoplasia of the thymus and parathyroid glands during embryonic development failure of T-cell maturation resulting in lymphopenia recurrent viral and fungal infections and tetany |
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CATCH 22?
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DiGeorge syndrome
C ardiac defects A bnormal facies T hymic hypoplasia C left palate H ypocalcemia microdeletion of chromosome 22 |
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Severe combined immunodeficiency disease (SCID) is also known as ___
deficiency of ___, which manifests as profound ____ and severed defects in ___ and ___ immunity. |
swiss type agammaglobulinemia
T and B cells; lymphopenia; humoral and cell-mediated |
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what causes SCID?
approximately 50% of autosomal recessive cases are caused by ____ which causes ____ |
can be autosomal recessive or X-linked
adenosine deaminase (ADA) deficiency; accumulation of substances that are toxic to lymphocytes |
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clinical manifestations of SCID?
anatomic manifestations of SCID? |
infections, malignancy, failure to thrive, and graft-versus-host disease
thymic hypoplasia and hypoplasia of lymph nodes and tonsils |
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What is Wiskott-Aldrich syndrome?
It is a ___ disorder |
immunodeficiency with thrombocytopenia and eczema
x-linked |
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What is hyper-IgM syndrome?
It is a ___ disorder results in increased propensity to ___ |
normal of elevated levels of IgM, but failure of isotype switching to IgG, IgA, or IgE
x-linked and autosomal recessive pyogenic infections |
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what causes AIDS?
Mechanisms of HIV infection: 1) The HIV virion expresses a cell surface protein, ___, with binding sites for ___. 2) Two recognition sites on gp120 for ____ and ____ are involved in entry of HIV into the cell. |
human immunodeficiency virus (HIV)
gp120; CD molecule on the surface of the CD4+ T cell coreceptors CCR5 and CXCR4 |
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Individuals that are____ are said to be highly resistant to some strains of HIV.
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homozygous for mutated CCR5 receptor
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What CD4+ cell types are targets for HIV infection?
How are monocytes and macrophages involved in HIV infection? How does HIV affect neural cells? |
monocytes, macrophages, dendritic cells, langerhans cells, and microglial cells of the CNS
they may function as reservoirs for HIV and vehicles for entry into the CNS directly by way of CD4 receptors or compete through the gp120 protein for neural receptor sites for neuroleukin |
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What happens after gp120 is bound to CD4 and HIV is internalized into the cell?
___ is integrated into the host genome HIV may remain in its ___ form for some time until activated by ____ or ____. |
DNA is synthesized by reverse transcription from genomic viral RNA
proviral DNA proviral; cytomegalovirus or Epstein-Barr virus (EBV) |
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What occurs during the latent period of HIV?
Where can the HIV virus be found? |
low-level virion production with infectivity
blood, semen, vaginal secretions, breast milk, and saliva |
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infection with HIV virus results in depletion of ___ cells.
___ ratio is also greatly reduced What does the loss of CD4+ helper T cells cause? Despite the ability to produce specific antibodies, patients will demonstrate ____ due to ____. |
CD4+ T cells
CD4+: CD8+ T cell ratio (often to less than 1) failure in humoral and cell-mediated hypersensitivity reactions hypergammaglobulinemia |
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What is Wiskott-Aldrich syndrome?
It is a ___ disorder |
immunodeficiency with thrombocytopenia and eczema
x-linked |
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What is hyper-IgM syndrome?
It is a ___ disorder results in increased propensity to ___ |
normal of elevated levels of IgM, but failure of isotype switching to IgG, IgA, or IgE
x-linked and autosomal recessive pyogenic infections |
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what causes AIDS?
Mechanisms of HIV infection: 1) The HIV virion expresses a cell surface protein, ___, with binding sites for ___. 2) Two recognition sites on gp120 for ____ and ____ are involved in entry of HIV into the cell. |
human immunodeficiency virus (HIV)
gp120; CD molecule on the surface of the CD4+ T cell coreceptors CCR5 and CXCR4 |
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Individuals that are____ are said to be highly resistant to some strains of HIV.
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homozygous for mutated CCR5 receptor
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What CD4+ cell types are targets for HIV infection?
How are monocytes and macrophages involved in HIV infection? How does HIV affect neural cells? |
monocytes, macrophages, dendritic cells, langerhans cells, and microglial cells of the CNS
they may function as reservoirs for HIV and vehicles for entry into the CNS directly by way of CD4 receptors or compete through the gp120 protein for neural receptor sites for neuroleukin |
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What happens after gp120 is bound to CD4 and HIV is internalized into the cell?
___ is integrated into the host genome HIV may remain in its ___ form for some time until activated by ____ or ____. |
DNA is synthesized by reverse transcription from genomic viral RNA
proviral DNA proviral; cytomegalovirus or Epstein-Barr virus (EBV) |
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What occurs during the latent period of HIV?
Where can the HIV virus be found? |
low-level virion production with infectivity
blood, semen, vaginal secretions, breast milk, and saliva |
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infection with HIV virus results in depletion of ___ cells.
___ ratio is also greatly reduced What does the loss of CD4+ helper T cells cause? Despite the ability to produce specific antibodies, patients will demonstrate ____ due to ____. |
CD4+ T cells
CD4+: CD8+ T cell ratio (often to less than 1) failure in humoral and cell-mediated hypersensitivity reactions hypergammaglobulinemia; polyclonal B cell activation |
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AIDS is characterized by severe immunodeficiency manifested by ___, ____, ____, and ____.
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opportunistic infection, kaposi sarcoma, B-cell non-Hodgkin lymphoma, and central and peripheral nervous system manifestations
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What is Kaposi sarcoma?
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rare neoplasm associated with human herpesvirus 8 which is also called Kaposi sarcoma herpesvirus (KSHV)
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What is HIV seropositivity?
What is AIDS marked by? |
begins soon after initial HIV infection, antibodies to the proteins coded by the genes of retroviral gag, env, and pol can be demonstrated, especially antibodies to gp120 and p24 proteins
HIV infection complicated by specified secondary opportunistic infection or malignant neoplasms |
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Autoimmunity is the apparent inability to ____
4 examples of autoimmune diseases? |
distinguish self from nonself
autoimmunue hemolytic anemia, Hashimoto thyroiditis, idiopathic adrenal atrophy, and connective tissue diseases |
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What are the 4 associations that are usually present in autoimmity?
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1) presence of autoantibodies
2) comorbidity with other autoimmune diseases 3) morphologic changes such as lymphoid follicle formation (Hashimoto thyroiditis) 4) association with specific HLA haplotypes |
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Autoimmunity
host antigens may recognize as ___ if modified by infection, inflammation, or complexing with a drug what happens when antigens that are usually isolated from the immune system are exposed to trauma or inflammation? example? |
non-self
they are recognized as foreign; thyroglobulin,, lens protein, and spermatozoa |
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what are specific autoantibodies?
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antibodies directed against host tissue, present in many autoimmune diseases
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What are three immunoregulation problems with autoimmunity?
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1) increase in helper T-cell function or decrease in suppressor T-cell function
2) nonspecific B-cell activation 3) thymic defects of B-cell defects |
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What are some examples of some autoimmune disorders that may be caused by genetics?
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Hashimoto thyroiditis, pernicious anemia, type 1 diabetes mellitus, and Sjorgen syndrome (all associated with other autoimmune disorders)
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What are the HLA antigen associations of Hashimoto thyroiditis and type 2 diabetes?
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Hashimoto- HLA-DR5 and HLA-B5
Type 2 diabetes- HLA-DR3 and HLA-DR4 |
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List the 6 connective tissue disorders that are autoimmune in origin.
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Systemic Lupus Erythematosus (SLE)
Progressive systemic sclerosis (PSS or scleroderma) Sjogren syndrome Polymyositis Mixed connective tissue disease (MCTD) Polyarteritis nodosa |
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SLE most often affects who?
What distinguishing things mark SLE? Which organ systems are usually affected? |
women (80%) of childbearing age
extensive immune complex-mediated inflammatory lesions involving multiple organ systems *kidney*, joints, skin, serous membranes, and lungs |
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SLE clinical manifestations
in joints? skin? Raynaud phenomenon? Serosa? endocarditis? spleen? kidney? eye? |
joints- arthralgia and arthritis
skin- characteristic butterfly rash over nose raynaud- vasospasm of fingers serosal inflammation (pericarditis and pleuritis) endocarditis- vegetation on mitral valve leaflet (Libman-Sacks) spleen- onion-skin appearance from fibrosis around splenic arterioles kidney- glomerular changes eye- cytoid bodies (yellow cotton wool-like lesions) |
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___ antibodies are found in almost all pts with SLE
best tests in SLE are for ____ DNA and ____. |
antinucleosome-specific autoantibody (ANA)
double stranded DNA and Sm (Smith) antigen |
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SLE tests give a biologic false positive for ___ due to ___.
What combination of antibodies is almost diagnostic of SLE? |
syphilis; anticardiolipins
antibodies specific for lupus: anti-ds-DNA and anti-Sm antigen |
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Progressive Systemic sclerosis (PSS) involves widespread _____ and ____ changes.
What does it affect? Who does it affect? |
fibrosis; degenerative
skin, GI tract (especially esophagus), heart, muscle, lung, and kidney yound women |
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PSS is marked by the presence of ___ in 1/3 of patients.
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ANA anti-Scl-70
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CREST syndrome?
CREST is ANA marked with ___ activity. |
C alcinosis
R aynaud phenomenon E sophageal dysfunction S clerodactyly T elangiectasia anticentromere |
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PSS characteristics of
face? hand? raynaud? visceral organs? |
hypertrophy of collagen fibers of the subcutaneous tissue, leading to tightening of facial skin and fixed facial appearance
hand- sclerodactyly- claw like appearance raynaud 75% pts, vasospasm in hand the main organ affected is esophagus with dysphagia and interstitial pulmonary fibrosis |
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Who does sjogren syndrome affect?
clinical manifestations...Triad of: eyes? mouth? always associated with a ___ |
women in their late middle age
keratoconjunctivitis sicca- dry eyes xerostomia- dry mouth CT disorder usually rheumatoid arthritis |
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what is Sicca syndrome
PSS affects which glands? what antibodies are present? |
xerostomia and keratoconjunctivitis alone
parotid and lacrimal ANAs including anti-SS-B and anti-SS-A |
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Polymyositis?
dermatomyositis? Who does it affect? What are they at at high risk for? |
chronic inflammatory process involving the proximal muscles of extremities (no cutaneous)
when the skin is involved with a reddish-purple rash on face and neck (heliotrope eyelids women malignancy |
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In Mixed CT disease (MCTD), ___ involvement is uncommon.
MCTD is characterized by ____. |
renal
specific ANAs- high-titer anti-nRNP and immunofluorescent speckled nuclear appearance |
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What characterizes Polyarteritis nodosa?
Occurs mainly in ___. What antigen is present? What is another name for this? |
segmental fibrinoid necrosis in the walls of small and medium arteries in any organ
men (in contrast to other CT diseases) Hep B antigen (30%) immune complex vasculitis |
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Amyloidosis is a group of disorders characterized by ___.
What is amyloid? It is characterized by abnormal ____. Amyloidosis always has a ___ configuration. |
deposition of amyloid
group of substances that share a common physical feature that can be formed by a number of different proteins) protein folding beta-pleated sheet configuration |
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Where can amyloid be found?
what does it look like and how does it stain with H&E? It is characteristically stained with the ___ dye, demonstrating ___ when viewed under polarized light. |
extracellular, proximal to basement membranes
amorphous eosinophilic appearance Congo red; apple green birefringence |
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What is another name for primary amyloidosis?
What is the cause? Where is amyloid deposition common? primary amyloidosis is is frequently associated with ____ |
immunocytic dyscrasia amyloidosis
deposition of amyloid fibrils derived from immunoglobulin light chains, referred to as AL (amyloid light chain) protein tissues of mesodermal origin (heart, muscle, tongue, kidney) plasma cell disorders |
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What is another name for secondary amyloidosis?
What is the cause? precursor? What is affected? sago spleen? secondary amyloidosis is a complication of ___ |
reactive systemic amyloidosis
deposition of the amyloid protein AA; precursor is SAA (serum amyloid associated protein) parenchymatous organs (kidney, liver, adrenals, pancreas, lymph nodes, and spleen) tapioca-like granules (perifollicular involvement) chronic inflammatory disease |
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beta 2-microglobulin derived amyloid is seen in patients on ____
transthyretin derived amyloid is found in _____ calcitonin-derived amyloid is found in ____ AL amyloid is found in ____ and is associated with ____ and ____. |
on long-term dialysis
senile amyloidosis and in a number of hereditary disorders medulary carcinoma of the thyroid primary amyloidosis ; multiple myeloma and other plasma cell disorders. |
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Goodpasture syndrome is marked by ______ due to antiglomerular basement membrane antibodies.
Also, Goodpasture syndrome is an example of a _________ reaction. |
linear immunofluoresence
type II complement dependent hypersensitivity |
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These antinuclear antibodies are indicative of what disorder?
1) Anti-Ro (or SS-A)? 2) anti-ribonucleoprotein (or RNP)? 3) anti-Jo-1? 4) anti-Scl-70? |
1) Sjögren syndrome
2) mixed connective tissue disease 3) polymyositis 4) scleroderma |