Unit5: Ch 12 (Porth's 5th Ed) - Disorders of the Immune Response Flashcards
- After years of going to different physicians with vague symptoms, a 55-year-old client
with a history of Hodgkin disease has been diagnosed with a secondary
immunodeficiency syndrome. The client asks the nurse what this means. The nurse
knows from the following list of characteristics that secondary immunodeficiency
disorders: Select all that apply.
A) may be inherited as a sex-linked trait.
B) usually develop later in life.
C) may be a result of chemotherapy being used to treat a cancer.
D) can result from frequent recurring Staphylococcus aureus infections.
E) can occur in a chronic obstructive pulmonary disease patient taking
corticosteroids daily.
Ans: B, C, E
Feedback:
Secondary immunodeficiency disorders develop later in life as a result of other
pathophysiologic states such as malnutrition, disseminated cancers, infection of the cells
of the immune system, and treatment with immunosuppressive drugs, such as
chemotherapeutic agents. Primary disorders may be congenital or inherited as
sex-linked, autosomal dominant, or autosomal recessive traits. Humoral (B-cell)
immunodeficiencies are primarily associated with recurrent infections like
Staphylococcus aureus.
- A 2-year-old girl has had repeated ear and upper respiratory infections since she was
born. A pediatrician has determined a diagnosis of transient hypogammaglobulinemia of
infancy. What is the physiological origin of the child’s recurrent infections?
A) The child’s immune system is unable to synthesize adequate immunoglobulin on
its own.
B) The child had a congenital absence of IgG antibodies that her body is only slowly
beginning to produce independently.
C) The child was born with IgA and IgM antibodies, suggesting intrauterine
infection.
D) The child lacks the antigen-presenting cells integral to normal B-cell antibody
production.
Ans: A
Feedback:
Transient hypogammaglobulinemia of infancy is characterized by inadequate
communication between B and T cells. IgG would be inherited through placental blood,
and an intrauterine infection is neither causative or nor synonymous with transient
hypogammaglobulinemia of infancy. The diagnosis does not include a lack of
antigen-presenting cells.
- A nurse is providing care for a 17-year-old boy who has experienced recurrent sinus and
chest infections throughout his life and presently has enlarged tonsils and lymph nodes.
Blood work indicated normal levels of B cells and free immunoglobulins but a lack of
differentiation into normal plasma cells. The boy is currently receiving intravenous
immunoglobulin (IVIG) therapy. What is the boy’s most likely diagnosis?
A) X-linked hypogammaglobulinemia
B) Transient hypoglobulinemia
C) Common variable immunodeficiency
D) IgG subclass deficiency
Ans: C
Feedback:
The lack of a terminal differentiation from B cells into plasma cells is the hallmark of
common variable immunodeficiency. Recurrent infections, enlarged lymph nodes and
tonsils, and IVIG therapy are also commonly associated.
- Which of the following patients is most likely to benefit from transplantation of thymic
tissue or major histocompatibility complex (MHC)-compatible bone marrow?
A) A 12-year-old girl with a history of epilepsy and low IgG levels secondary to
phenytoin use
B) A 7-year-old boy whose blood work indicates decreased IgA and IgG with
increased IgM
C) A 6-year-old boy whose pre-B cells are incapable of translation to normal B cells
D) A 9-year-old girl who has a diagnosis of IgA deficiency
Ans: B
Feedback:
Decreased IgA, IgE, and IgG with increased IgM levels are characteristic of X-linked
immunodeficiency with hyper-IgM, a primary cell-mediated immunodeficiency that
would respond to thymic tissue transplantation and MHC-compatible bone marrow.
Low IgG levels secondary to phenytoin use, X-linked hypogammaglobulinemia, and
selective IgA deficiency are unlikely to be treated with the T-cell–focused treatments
like thymic tissue transplantation and MHC-compatible bone marrow.
- An 8-week-old boy has been recently diagnosed with a severe combined
immunodeficiency (SCID). His parents have performed a significant amount of research
on the Internet and have brought a large amount of material to discuss with their care
provider. Which of the following statements best reflects an accurate understanding of
their son’s health situation?
A) “We read that gene therapy could cure our son; we’d like you to look into that
option.”
B) “Our son likely has a deficiency of B lymphocytes and can’t produce antibodies.”
C) “We feel guilty, because dietary and environmental factors have been shown to
contribute to SCID”
D) “The antibodies that our son produces are mismatched to the infections that he
was born with and encounters.”
Ans: B
Feedback:
The pathophysiology of SCID involves normal B cells but a lack of antibody production
because of inadequate T-cell help. Gene therapy is not yet a realistic treatment option,
and the disease has a genetic basis. Antibodies are not incorrect but rather inadequate in
number.
- A 1-year-old child who has experienced low platelet counts and bacterial susceptibility
has been admitted to a pediatric medical unit of a hospital for treatment of
Wiskott-Aldrich syndrome. The nurse who has admitted the child to the unit would
anticipate which of the following short-term treatment plans?
A) Transfusion of clotting factors XII and XIII and serum albumin; splenectomy
B) Neutropenic precautions; fresh frozen plasma transfusions; treatment of
gastrointestinal symptoms
C) Intravenous immunoglobulin (IVIG) treatment; thyroidectomy
D) Treatment of eczema; management of bleeding; bone marrow transplant
Ans: D
Feedback:
Common interventions for Wiskott-Aldrich syndrome involve controlling eczema,
managing bleeding due to low platelets, and ultimately bone marrow transplant. The
other noted interventions are not associated with the treatment of Wiskott-Aldrich
syndrome.
- A nurse has just learned that her child has a life-threatening complement disorder
known as hereditary angioneurotic edema (HAE). Due to deficiency in C1-INH, the
nurse needs to be prepared for which possible life-threatening clinical manifestation?
A) Bulging eyeballs
B) Swelling of the airway
C) Compressed carotid arteries
D) Compression of brachial nerves
Ans: B
Feedback:
HAE is a rare, life-threatening complement disorder that results from deficiency of
C1-inhibitor (HAE-C1-INH). It is an inherited autosomal dominant trait that causes
mutation in the 11th chromosome. Deficiencies in C1-INH result in uncontrolled release
of vasoactive substances that promote vascular permeability. The net result is the
development of swelling in the subcutaneous tissues of the extremities, face/torso, or
upper airway and GI tract. Laryngeal edema is a life-threatening manifestation that can
lead to complete airway obstruction and death without interventions.
- A 4-year-old boy presents with a chronic cough and swollen lymph nodes. His records
show that he has been given antibiotics several times in the past year with limited
success, most recently for a liver abscess, and that he also has a recurring fungal skin
condition. Which of the following is his most likely diagnosis?
A) Selective IgA deficiency
B) A deficiency in IgG2 subclass antibodies
C) Chronic granulomatous disease
D) Ataxia–telangiectasia
Ans: C
Feedback:
Chronic granulomatous disease, because it affects phagocytic function, increases
susceptibility to soft tissue infections, particularly of the skin, lungs, lymph nodes, and
liver. Selective IgA deficiency and deficient IgG2 subclass antibodies can predispose
people to infection, but those infections respond readily to antibiotic treatment.
Ataxia–telangiectasia can cause skin and liver problems, but its primary manifestations
are ataxia and telangiectasia
- A patient diagnosed with a primary immunodeficiency disorder has asked his siblings to
be tested as possible stem cell donors. When discussing this procedure with his family,
the nurse emphasizes that stem cells can be harvested from: Select all that apply.
A) bone marrow.
B) peripheral blood.
C) skin tissue harvesting.
D) mouth swabs.
E) tears
Ans: A, B
Feedback:
Hematopoietic stem cells can be harvested from either the bone marrow or the
peripheral blood and also from the umbilical cord blood. From sibling donors, the
results are effective with improved survival in approximately 90% of people.
- When explaining what is occurring when their child has an acute bronchial asthma
attack, the nurse will emphasize that which mediator is primarily responsible for the
bronchial constriction?
A) Tree pollen
B) Mold dust
C) Histamine
D) T-lymphocyte proliferation
Ans: C
Feedback:
Asthma response begins within 5 to 30 minutes of exposure to an allergen. It is
mediated by mast cell degranulation and the release of preformed and/or enzymatically
activated mediators. These mediators include histamine, serotonin, and acetylcholine.
Histamine is the most recognized mediator of type I hypersensitivity reactions and
ultimately results in bronchial constriction.
- A male elementary school student has a severe allergy to peanuts and is displaying the
signs of anaphylactic shock after inadvertently eating a peanut-containing candy bar.
Which of the following statements best captures the boy’s current status and preferred
treatment?
A) He is experiencing shortness of breath caused by potent vasoconstriction that can
be relieved by epinephrine injection.
B) He is approaching vascular shock and developing edema due to actions of IgE
antibodies, situations that can be reversed by administration of epinephrine.
C) His mast cells and basophils have been sensitized, but systemic effects can be
mitigated by administration of bronchodilators.
D) He is likely in a primary- or initial-stage allergic response that can be relieved by
antihistamine administration
Ans: B
Feedback:
Anaphylactic reactions are often accompanied by vascular shock and edema, and the
normal treatment is epinephrine injection. Symptoms are caused by vasodilation, not
vasoconstriction; bronchodilators are not the ideal treatment.
- A 40-year-old woman who experiences severe seasonal allergies has been referre d by
her family physician to an allergist for weekly allergy injections. The woma n is
confused as to why repeated exposure to substances that set off her allergie s would
ultimately benefit her. Which of the following phenomena best captures the rationale f or
allergy desensitization therapyN?URSINGTB.COM
A) Repeated exposure to offending allergens binds the basophils and mast cells that
mediate the allergic response.
B) Allergens in large, regular quantities overwhelm the IgE antibodies that med iate
the allergic response.
C) Repeated exposure stimulates adrenal production of epinephrine, mitigating the
allergic response.
D) Injections of allergens simulate production of IgG, combining with the antigens to
prevent activation of IgE antibodies.
Ans: D
Feedback:
Repeated exposure to allergens causes an increase in IgG, which binds with antigens
before they can stimulate IgE. It does not bind mast cells or basophils, nor does it
overwhelm the IgE antibodies to stimulate epinephrine production.
- A 24-year-old woman has gone to the OB-GYN clinic for her first visit since she found
out she was pregnant. The clinician tested her blood type along with the usual prenatal
testing. On a follow-up visit, the woman was told that she is Rh negative. When asked
what that means for her baby, the nurse explains that Rh-negative women lack RhD
antigens on their erythrocytes but produce anti-D antibodies. As a result of this blood
type,
A) “If you and your baby have mismatched blood, it can invoke anaphylaxis in the
baby.”
B) “If the types are incompatible, severe antibody-mediated inflammation occurs.”
C) “If blood types do not match, the baby’s liver will produce extra cells to replace
RBCs needed to oxygenate organs.”
D) “If the fetus is Rh positive, maternal anti-D antibodies can coat fetal RBCs
resulting in severe anemia.
Ans: D
Feedback:
In utero, the development of erythroblastosis fetalis (Rh incompatibility) results when
Rh-negative women produce anti-D antibodies. In Rh-positive fetus, maternal anti-D
antibodies will coat fetal RBCs containing RhD, allowing them to be removed from the
fetal circulation by macrophage- and monocyte-mediated phagocytosis (destroying
RBCs). RBCs are produced in the bone marrow, not the liver; therefore, answer C is
incorrect.
- A 67-year-old patient diagnosed with myasthenia gravis will likely display which
clinical manifestations as a result of autoantibodies ultimately blocking the action of
acetylcholine, resulting in destruction of the receptors?
A) Weakness of the eye muscles; difficulty in swallowing and slurred speech;
impaired gait
B) Tremor of hands/arms; rigidity of the arms; shuffling gait
C) Short-term memory lapses; problems with orientation; a lack of drive or initiative
D) Facial droop; slurred speech; weakness on one side of the body
Ans: A
Feedback:
Myasthenia gravis ultimately results in destruction of receptors in the neuromuscular
junction leading to a decrease in neuromuscular function. Answer B relates to s/s of
Parkinson disease; answer C relates to Huntington disease; and answer D relates to
classic s/s of CVA.
- Following a spider bite she received while camping, a 20-year-old female presented to
the emergency department with rash, edema, and fever and was subsequently diagnosed
with serum sickness. Which of the following statements best conveys the physiological
rationale for the broad systemic effects of this event?
A) The woman is experiencing diffuse tissue necrosis as a consequence of an Arthus
reaction.
B) Antigen–antibody complexes have been deposited in a variety of locations
throughout the body.
C) Antibody binding to specific target cell receptors is bringing about a change in
cell function.
D) Deposited antibodies are activating her complement system.
Ans: B
Feedback:
Serum sickness is characterized by the deposition of antigen–antibody complexes in
blood vessels, joints, heart, and kidney tissue. The deposited complexes activate
complement, increase vascular permeability, and recruit phagocytic cells, all of which
can promote focal tissue damage and edema. Serum sickness is not synonymous with an
Arthus reaction. Antibody binding to specific target cell receptors causing a change in
cell function is characteristic of antibody-mediated cellular dysfunction. Serum sickness
is not associated with the activation of the complement system
