Under normal conditions, the ____ response deters or prevents disease.
immune
____ can be defined as an abnormality in the immune system that renders a person susceptible to diseases normally prevented by an intact immune system.
Immunodeficiency
The ____ immune system is composed of the phagocytic leukocytes, natural killer (NK) cells, and complement proteins.
innate
The ____ immune response is composed mainly of T and B cells and responds to infections more slowly, but more specifically, than the innate immune system.
adaptive
The adaptive immune system is further divided into the ___ and _____ immune systems.
humoral, cellular
A large number of primary immunodeficiency diseases have been mapped to the ____ chromosome.
X
Defects in humoral immunity increase the risk of recurrent _____ infections.
pyogenic
During the first few months of life, infants are protected from infection by IgG antibodies that originate in _____ circulation during fetal life.
maternal
Of all the primary immunodeficiency diseases, those affecting _____ production are the most frequent.
antibody
Abnormal immunoglobulin loss can occur with chronic _____ disease; because of abnormal glomerular filtration, patients lose serum IgA and IgG in their urine.
kidney
Secondary humoral immunodeficiencies can also result from a number of ____ including chronic lymphocytic leukemia, lymphoma, and multiple myeloma that interfere with normal immunoglobulin production.
malignancies
T cells can be functionally divided into two subtypes: ____ and ____ T cells.
CD4+ helper, CD8+ cytotoxic
Collectively, _____ protect against fungal, protozoan, viral, and intracellular bacterial infections; control malignant cell proliferation; and are responsible for coordinating the overall immune response.
T lymphocytes
Disorders that affect both B and T lymphocytes, with resultant defects in both humoral and cell-mediated immunity, fall under the broad classification of _____ syndrome.
combined immunodeficiency
In _____, genetic mutations lead to absence of all T and B cell function and, in some cases, a lack of NK cells.
severe combined immunodeficiency (SCID)
SCID is more commonly found in _____, as it is X linked.
boys
Hereditary angioneurotic edema is a form of _____ deficiency.
complement
Chronic cirrhosis of the liver would reduce the production of complement proteins; this type of deficiency would be classified as _____.
secondary
Chediak-Higashi syndrome is an abnormality of _____ of phagocytes.
degranulation
Chronic granulomatous disease is a group of inherited disorders that greatly reduce or inactivate the ability of phagocytic cells to produce the _____.
respiratory burst
_____ disorders refer to excessive or inappropriate activation of the immune system.
Hypersensitivity
Type I hypersensitivity reactions to antigens is referred to as ______.
allergic reactions
____ is a systemic life-threatening hypersensitivity reaction characterized by widespread edema, vascular shock secondary to vasodilation, and difficulty breathing.
Anaphylaxis
Persons with ____ allergic conditions tend to have high serum levels of IgE and increased numbers of basophils and mast cells.
atopic
Allergic ___ is characterized by symptoms of sneezing, itching, and watery discharge from the nose and eyes.
rhinitis
There are three different types of antibody-mediated mechanisms involved in ____ reactions: Opsonization and complement- and antibody receptor-mediated phagocytosis, complement- and antibody receptor-mediated inflammation, and antibody-mediated cellular d
type II
____ mediated destruction of cells that are coated with low levels of IgG antibody and are killed by a variety of effector cells, which bind to their target by their receptors for IgG and cell lysis occurs without phagocytosis.
Antibody-dependent cellular cytotoxicity
___ hypersensitivity reactions are responsible for the Vasculitis seen in certain autoimmune diseases, such as systemic lupus erythematosus (SLE), or the kidney damage seen with acute glomerulonephritis.
Type III
____ sickness is a systemic immune complex disorder that is triggered by the deposition of insoluble antigen-antibody complexes in blood vessels, joints, heart, and kidney tissue.
Serum
A term used by pathologists and immunologists to describe localized tissue necrosis caused by immune complexes is the ______.
Arthus reaction
Hypersensitivity reactions that are mediated by specifically sensitized T lymphocytes are divided into two basic types: direct cell-mediated cytotoxicity and delayed-type hypersensitivity, and generally classified as ______.
type IV reactions
Allergic _______ denotes an inflammatory response confined to the skin that is initiated by re-exposure to an allergen to which a person had previously become sensitized.
contact dermatitis
A major barrier to ____ is the process of rejection in which the recipient's immune system recognizes the graft as foreign and attacks it.
transplantation
Transplanted tissue can be categorized as an _____ graft if donor and recipient are the same person, _____ graft if the donor and recipient are identical twins, and _____ if the donor and recipient are related or unrelated but share similar HLA types.
autologous, syngeneic, allogeneic
_____ occurs when immunologically competent cells or precursors are transplanted into recipients who are immunologically compromised.
graft-versus-host disease
____ diseases represent a group of disorders that are caused by a breakdown in the ability of the immune system to differentiate between self- and non-self-antigens.
autoimmune
The ability of the immune system to differentiate self from nonself is called _____.
self-tolerance
Loss of self-tolerance with development of _____ is characteristic of a number of autoimmune disorders.
autoantibodies
DiGeorge syndrome
Partial or complete failure of development of the thymus and parathyroid glands
Secondary immunodeficiency
Acquired later in life
Hyper-IgM syndrome
Low IgG and IgA levels, high IgM concentrations
X-linked agammaglobulinemia
Essentially undetectable levels of all serum immunoglobulins
Selective IgA deficiency
In levels of serum and secretory IgA
Adenosine deaminase deficiencies and T-cell cytokine receptor mutations
Cause of SCID
Transient hypogammaglobulinemia of infancy
Repeated bouts of upper respiratory and middle ear infections
Ataxia-telangiectasia
Lymphopenia and a decrease in the ratio of CD4+ helper T cells to CD8+ suppressor T cells
Common variable immunodeficiency
the terminal differentiation of mature B cells to plasma cells is blocked
Immunoglobulin G subclass deficiency
decreases in one or more of IgG subgroups
Wiskott-Aldrich syndrome
Susceptible to infections caused by encapsulated microorganisms
Type I hypersensitivity reaction
IgE mediated disorders
Type II hypersensitivity reaction
antibody-mediated disorders
Type III hypersensitivity reaction
Complement-mediated immune disorders
Type IV hypersensitivity reaction
T-cell mediated disorders
Put the normal sequence of actions of a polymorphonuclear phagocyte in order.
a. Phagocytosis
b. Kill the ingested pathogens
c. Chemotaxis
d. Generate microbidcidal substances
e. Adherence
1. Chemotaxis
2. Adherence
3. Phagocytosis
4. Generate microbidcidal substances
5. Kill the ingested pathogens
What is the difference between a primary and a secondary immunodeficiency?
A primary deficiency or immunodeficiency is congenital or inherited. A secondary immunodeficiency is acquired.
Why does it take up to 6 months for the symptoms of a primary immunodeficiency to show up?
During the first few months of life, infants are protected from infection by IgG antibodies that have been transferred from the maternal circulation during fetal life. An infant's level of maternal IgG gradually declines over a period of approximately 6 m
Explain how a patient can become sensitized to an allergen (antigen) in a type I hypersensitivity reaction.
Type I hypersensitivity reactions begin with mast cell or basophil sensitization. During the sensitization or priming stage, allergen-specific IgE antibodies attach to receptors on the surface of mast cells and basophils. With subsequent exposure, the sen
Compare the direct cell-mediate cytotoxicity of type IV hypersensitivity reactions with the delayed-type hypersensitivity reactions.
In direct cell-mediated cytotoxicity, CD8+ cytolytic T lymphocytes (CTLs) directly kill the antigen-presenting target cells. In viral infections, CTL responses can lead to tissue injury by killing infected target cells even if the virus itself has no cyot
What is SCID?
Severe combined immunodeficiency (SCID) is the result of genetic mutations that lead to absence of all T- and B-cell function and, in some cases, lack of natural killer cells. Affected infants have a disease course that resembles AIDS, with failure to thr
Infants are born with a passive immunity that occurs when immunoglobulin antibodies cross the placenta from the maternal circulation prior to birth. Which immunoglobulin is capable of crossing the placenta?
IgG. During the first few months of life, infants are protected from infection by IgG antibodies that have been transferred from the maternal circulation during fetal life. IgA, IgM, IgD, and IgE do not normally cross the placenta.
Drug-induced secondary hypogammaglobulinemia is considered reversible. Which drugs produce hypogammaglobulinemia?
Phenytoin, Corticosteroids, Carbamazepine, and Disease-modifying antirheumatic drugs. Medications that cause reversible secondary hypogammaglobulinemia include the disease-modifying antirheumatic drugs, corticosteroid agents and the antiepileptic drugs, p
Primary cell-mediated disorders of the immune system cause severe problems with infections. Children with these disorders rarely survive beyond childhood without a bone marrow transplant. What is a disease that involves primary cell-mediated disorders of
DiGeorge Syndrome. In general, persons with cell-mediated immunodeficiency disorders have infections or other clinical problems that are more severe than antibody disorders. Children with defects in this branch of the immune response rarely survive beyond
Combine immunodeficiency syndrome is a disorder in which both B and T lymphocytes are affected. This results in defects in both humoral and cell-mediated immunity. What could be the cause of this disorder?
A single mutation in any gene that influences major histocompatibility antigens. Disorders that affect both B and T lymphocytes with resultant defects in both humoral and cell-mediated immunity fall under the broad classification of combined immunodeficie
Combined immunodeficiency (CID) is distinguished by low, not absent, T-cell function. These diseases are usually associated with other disorders and arise from diverse genetic causes. What disease is considered a CID?
Ataxia-telangiectasia. Ataxia-telangiectasia is a complex syndrome of neurologic, immunologic, endorocinologic, hepatic, and cutaneous abnormalities. Pierre-Robin syndrome, Angelman syndrome, and Adair-Dighton syndrome are not immunologic deficiencies.
The immune system typically responds to invaders of all types in our body. However, it can also cause tissue injury and disease. What is this effect called?
Hypersensitivity action. Disorders caused by immune responses are collectively referred to as hypersensitivity reactions. Antigens cause allergic reactions. Mediator response action and allergen stimulating reaction have nothing to do with hypersensitivit
Some people are so sensitive to certain antigens that they react within minutes by developing itching, hives, and skin erythema, followed shortly thereafter by bronchospasm and respiratory distress. What is this commonly known as?
Anaphylactic reaction. Anaphylaxis is a systemic life-threatening hypersensitivity characterized by widespread edema, vascular shock secondary to vasodilation and difficulty breathing. It is not called an antigen reaction, neither is it called an Arthus r
A systemic immune complex disorder that is caused by insoluble antigen-antibody complexes being deposited in blood vessels, the joints, the heart, or kidney tissue is called what?
Serum sickness. Serum sickness is a systemic immune complex disorder that is triggered by the deposition of insoluble antigen-antibody (IgM, IgG, and occasionally IgA) complexes in blood vessels, joints, heart, and kidney tissue. This is not anti-immune d
The incidence of latex allergy is skyrocketing because of diseases such as HIV. It is known that the use of latex examining gloves has played a major role in the increasing incidence of latex allergy. What plays a significant role in the allergic response
Cornstarch powder used inside the gloves. Cornstarch powder is applied to the gloves during the manufacturing process to prevent stickiness and give the gloves a smooth feel. The cornstarch glove powder has an important role in the allergic response. Late
A transplant reaction that occurs immediately after transplantation is caused by ____ antibodies that are present.
A transplant reaction that occurs immediately after transplantation is caused by antidonor antibodies that are present. When preformed antidonor antibodies are present, rejection occurs immediate after transplantation.
It has been postulated that an autoimmune disease needs a "trigger event" for it to clinically manifest itself in a body. What are these "trigger events" thought to be?
A microorganism or virus, A self-antigen from a previously sequestered body tissue, or a chemical substance. Because autoimmunity does not develop in all persons with genetic predisposition, it appears that other factors such as a "trigger event" interact