Formative Quiz Review 2 Flashcards

1
Q

You have a patient with repeated infections and normal T cell function. However, you determine that the patient has agammaglobulinemia. Which one of the following tests would determine if this immune deficiency is due to an absence of B cells?

Radioimmunoassay

Flow cytometry

ELISA

Immunofixation

Coombs test

A

A. To confirm that an agammaglobulinemia is due to an absence of B cells, one needs to actually measure B cell number in the periphery. Radioimmunoassay cannot accomplish this goal.
B. Correct. Flow cytometry is a routine test for measuring the number, percentage or ratio of various cell subsets. It is generally performed on peripheral blood samples, but can also be used to measure cell frequencies in suspensions of dispersed lymph node or spleen cells. B cells are CD19+.
C. To confirm that an agammaglobulinemia is due to an absence of B cells, one needs to actually measure B cell number in the periphery. Enzyme-linked immunoassays (ELISA), which are similar to radioimmunoassay. cannot accomplish this goal.
D. To confirm that an agammaglobulinemia is due to an absence of B cells, one needs to actually measure B cell number in the periphery. Immunofixation, which is used to determine the isotype of an antibody, cannot accomplish this goal.
E. To confirm that an agammaglobulinemia is due to an absence of B cells, one needs to actually measure B cell number in the periphery. The Coombs test, which detects autoantibodies on erythrocytes, cannot accomplish this goal

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2
Q

A 54-year-old man presents with fever, a severe productive cough, and wheezing. Chest X-ray reveals infiltration in the lower right lobe. The patient was treated empirically with antibiotics without clinical improvement. Based on the results of his sputum sample, you conclude the patient needs to receive piperacillin. Within a couple days, the patient responds to therapy as seen by a reduction in fever and less coughing and wheezing. Seven days after starting piperacillin, the patient develops a fever (39.5°C), uticaria, and purpuric lesions. The patient also complains of stiffness in the joints. Which of the following Gell and Coombs classifications best describes the pathophysiology behind the phenomena seen after piperacillin treatment?

I

II

III

IV

This is not immune-mediated tissue damage.

A

III

This is serum sickness. Note the timing of the reaction, seven days after starting therapy. This is too late for Type I.

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3
Q

A delayed-type hypersensitivity reaction in skin to tuberculin PPD is delayed 24-48 hours, primarily because:

B cells need time to form specific antibodies, which lead to the hypersensitivity reaction.

Complement component C5a takes time to exert its chemotactic effect on neutrophil.

Mycolic acids present in Mycobacteria temporarily inactivate the vasoactive amines.

Prostaglandins and leukotrienes have difficulty diffusing from a granuloma.

T cell recruitment to the site of injury takes time to occur.

A

T cell recruitment to the site of injury takes time to occur.

“Yep. That’s why they call it delayed type hypersensitivity.”

This is actually what they said for the response lol

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4
Q

Which of the following is required for mast cell degranulation during the elicitation phase of Type I hypersensitivity?

IL-4 production

NFkB activation

Sustained cAMP levels

PLA2 activation

PLCγ1 activation

A

PLCγ1 activation

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5
Q

An adolescent with a history of childhood upper respiratory tract infections and diarrhea continues to have recurring sinusitis, which is attributed to Pseudomonas, a gram-negative bacterium. Laboratory tests over the past 5 years have repeatedly indicated that he has normal T cell numbers (based on CD3, CD4 and CD8), normal serum complement levels and activity, and normal serum IgM and IgG levels for his age. Isohemagglutinin titers are normal. Neutrophil numbers and function (chemotaxis, opsonophagocytosis and respiratory burst) are all normal. His IgG antibody response to tetanus toxoid challenge is also within the normal range. He shows 4 reactions in delayed hypersensitivity skin tests against several common fungal antigens. What is your therapy for this patient?

Antibiotics only

Antibiotics and IVIG

Antibiotics, IVIG and stem cell transplantation

Antibiotics and interferon-γ

Antibiotics and IL-2

A

A. Correct. This patient shows no evidence of an underlying immune defect, despite extensive laboratory and clinical tests for each of the major types of immune deficiencies. Prudent treatment would be antibiotic therapy for the infection.
B. Antibiotics would be helpful for this patient. While one might argue that IVIG is reasonably safe, in the absence of evidence that it would help this particular patient, it should not be given. All drugs have potential side effects, including in this case the rare anaphylactoid reaction.
C. Antibiotics would be helpful for this patient. However, stem cell transplantation should be avoided in all antibody deficiencies unless the more conservative therapy (IVIG) fails. Moreover, while one might argue that IVIG is reasonably safe, in the absence of evidence that it would help this particular patient, it should not be given. All drugs have potential side effects, including in the case of IVIG the rare anaphylactoid reaction.
D. Antibiotics would be helpful for this patient. However, while interferon-γ does enjoy some success in the treatment of phagocytic cell deficiencies, there is no evidence of such a deficiency in this patient.
E. Antibiotics would be helpful for this patient. However, IL-2 is not recommended because there is no evidence of an IL-2 deficiency or a Th deficiency.

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6
Q

By which of the following mechanisms can IgG regulate immune responses?

Binding FcgR on B cells to inhibit B cell activation

Binding FcgR on B cells to promote B cell activation

Binding FcgR on macrophages to prevent IL-10 production

Binding to soluble antigen to inhibit opsonization

Binding to soluble antigen to inhibit complement activation

A

A. Correct
B. FcgR on B cells is an inhibitory receptor
C. FcgR on macrophages induces IL-10 production.
D. IgG is an effective opsonizing agent.
E. IgG promotes complement.

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7
Q

In the accompanying high power photomicrograph of infarcted myocardium, which one of the following estimates of the age of the infarct is most likely correct?

1-2 hours

4-12 hours

2-3 days

10 days

2 weeks

1 year

A

10 days - The tissue appears loose and edematous. Dead myofibrils are still visible. The infiltrate consists of large cells with abundant cytoplasm (macrophages). This puts it at around 7-10 days, according to Robbins Table 12-5.

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8
Q

A 25-year old woman starts out on a long walk in the woods to admire the fall colors. After a short time, she begins wheezing and develops shortness of breath. Which of the following is most likely responsible for these symptoms:

C3

CD8+ T cells

Factor B

IgE

Neutrophils

A

A. C3 becomes activated when 1) it directly binds a microbe (Alternative pathway), 2) when Ab-Ag complexes activate the classical pathway, or 3) when MBL binds a microbe (lectin pathway). The woman is most likely suffering from a hay fever-like syndrome.
B. CD8+ T cell-mediated responses to not become activated “after a short time”.
C. Factor B is only activated via the alternative pathway, which is unlikely to be causing the symptoms in this patient.
D. Correct
E. The woman is most likely suffering from a hay fever-like syndrome, a type I hypersensitivity

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9
Q

A 4-year-old girl presents with an infection with the intracellular bacterium Mycobacterium avium intracellulare. She is found to have a rare genetic deficiency in a cytokine receptor. Her T cells fail to produce IFN-g when stimulated with mycobacterial antigens and antigen-presenting cells in vitro. Which one of the following cytokine receptors most likely is defective in this patient?

IL-1 receptor

TNF-a receptor

IL-4 receptor

IL-8 receptor

IL-12 receptor

A

Of the cytokine receptors listed, only the IL-12 receptor is a co-inducer of IFN-g production by T cells. MAI would be expected to activate macrophages and DCs to produce IL-12, but this patient lacks the IL-12 receptor and her T cells will not respond to IL-12 produced in response to the pathogen. Without that signal, IFN-g is not produced and the patient is at risk for infections with intracellular bacteria.

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10
Q

A 22-year-old woman returned to the United States from a trip to Mexico. She suddenly became ill, with nausea and vomiting. She underwent a needle biopsy of the liver. Later, serology was found to be positive for Hepatitis A Virus. A representative section of her liver biopsy is shown in the accompanying photomicrograph. Which one of the following interpretations of the eosinophilic bodies denoted by the arrows marked “C” is most likely CORRECT?

Acute inflammatory cells

Apoptotic bodies

Bile ducts

Chronic inflammatory cells

Mallory (“alcoholic”) hyaline

Viral inclusion bodies

A

The eosinophilic structures are a result of the ordered cell death of apoptosis, and are called apoptotic bodies. They were formally given specific, eponymic names in different tissue (e.g., Councilman bodies in the liver, Civatte bodies in the skin), but now all are simply called apoptotic bodies. They are usually not accompanied by acute inflammation, unlike necrosis.

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11
Q

Which subset of myeloid-derived cells is likely involved in contact dermatitis (e.g., poison ivy rash)?

Interdigitating reticulocytes

Kuppfer cells

Langerhans cells

Mesangial cells

Microglia

A

Langerhans cells of the epidermis (not to be confused with Langhans Giant cells), which are essentially macrophages, are required for presentation of antigen and the delayed type hypersensitivity reaction.

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12
Q

A deficiency in Factor I would have what observed?

A

Deficiency of Factor I would lead to depressed C3 levels due to a lack of control of the alternative pathway positive amplification loop.

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13
Q

In the accompanying photomicrograph of a tuberculous granuloma, the multi-nucleated giant cell in the middle is derived from which one of the following cells?

Macrophage

Lymphocyte

Neutrophil

Plasma cell

T cell

A

Macrophage

The multinucleated Langhans giant cell has macrophage cell surface markers, and is the result of cell fusion, due to cytokine stimulation, which happens because the mycobacteria cannot be digested by the cell, resulting in further stimulation.

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14
Q

An elderly woman was transferred from a long-term care facility to your hospital with a urinary tract infection. You treat her with IV ampicillin, ceftriaxone and fluids. The next day, she suddenly develops severe hypotension, tachycardia, tachypnea and loses consciousness. She has no history of adverse effects to these antibiotics. Which of the following inflammatory mediators most likely contributed to her acute systemic inflammatory response?

IL-2

IL-4

C1q

Histamine

TNF-a

A

Among the possible diagnoses of this patient, sepsis seems quite likely due to the symptoms and the common nature of this disease. Sepsis is mediated by inflammatory cytokines and other mediators, and TNF is among the most important.

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15
Q

Intravenous immunoglobulin therapy is most appropriate for which of the following disorders?

IgG multiple myeloma

Btk deficiency

IgA deficiency

C1 deficiency

Chronic granulomatous disease

A

Btk Deficiency

IVIG is most often prescribed for antibody deficiencies. The drug does not benefit myeloma patients due to their high turnover of Igs. IVIG is considered too risky to give to IgA deficiency, although this is controversial.

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16
Q

An 18-month-old boy presents to your clinic for the eighth time with an upper respiratory tract infection. Each time, the patient’s infection responded favorably to antibiotic therapy. However, after discontinuing the antibiotics, the infection returned. A CBC revealed white blood cell and lymphocyte counts in the normal range for his age. An analysis of the patient’sat there was less than 3 mg/dL each of IgM, IgG, and IgA. Which one of the following proteins is MOST LIKELY to be deficient in this patient?

Btk

CD18

CD40L

CIITA

ZAP-70

A

Btk

The number of infections suggests an immunodeficiency. The patient responds to antibiotics, but the infection recurs upon withdrawal of the drugs. He is unable to mount a sufficient immune response to completely clear the infection. He is extremely low antibody levels, suggesting a B cell defect. A B cell deficiency is not obvious on a CBC; a B/T panel would be needed. He is a boy, so Btk is possible. None of the others would have low levels of all Igs. A T cell deficiency would not impair IgM.

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17
Q

Deficiency of which of the following proteins would result in an immune deficiency that is similar to C3 deficiency?

Btk

IFNγ

IL-10

IL-12

ZAP-70

A

C3 is downstream of antibodies (the classical pathway) or innate immune pathways (MBL pathway). The alternative pathway functions in both the classical and MBL pathways. Btk-deficient patients cannot produce antibodies and thus cannot activate the classical pathway. IL-10 is an inhibitory molecule and these patients would have autoimmunity. IFNγ, IL-12, and ZAP-70 are T cell molecules and would have a different clinical presentation.

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18
Q

What Ab isotypes would be impaired with a Btk deficiency?

A

There would be a deficiency in all antibody isotypes

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19
Q

Mutations in either Rag-1 or Rag-2 result in immunodeficiencies in human beings. Which one of the following cell types would show NORMAL NUMBERS in a patient with a Rag-1 deficiency?

CD3+ cells with a CD4 coreceptor

CD19+ cells

Single-positive thymocytes

Lymphocytes with a coreceptor specific for C3d

Cells with an inhibitory receptor specific for MHC class I

A

Cells with an inhibitory receptor specific for MHC class I

These are NK cells and are not dependent on MHC class I presentation

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20
Q

Which one of the following laboratory tests is the most important to order on a 2-month-old child who presents with diarrhea and thrush?

Skin test for reactivity to fungal antigens

Serum IgG levels

Serum complement levels

Salivary IgA levels

ELISA for anti-HIV-1 antibodies

A

A. At this age a child may not show reactivity to fungal antigens even though he has been exposed to the antigens (thrush is a mucocutaneous infection by the yeast Candida albicans). A negative test result is therefore inconclusive.
B. The IgG in the serum of a 2-month-old child is primarily derived from the mother, being transported during gestation across the placenta. Thus, measuring its concentration will tell you very little about the immune status of the child.
C. Thrush is also known as mucocutaneous candidiasis (a yeast infection). Complement plays only a minor role in host defense against that opportunistic pathogen.
D. Although knowing that thrush is another name for a yeast infection of the mucous membranes of the gastrointestinal tract (including the oral cavity), one cannot conclude that mucosal IgA antibodies play a role in host defense at this early age. Normal children do not begin to synthesis significant quantities of secretory IgA until later in life and only reach adult levels in their early teens.
E. Correct. The finding of an opportunistic yeast infection in a child at this early age implies a T cell deficiency. The most common cause in this age group is neonatal AIDS.

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21
Q

A 16-year-old woman was given penicillin for an apparent “Strep throat”. She soon developed a reddish-appearing skin rash, which was firm, when palpated. A biopsy was performed. A representative section of the biopsy is shown in the accompanying photomicrograph. Which one of the following is the best diagnosis?

Angiosarcoma

Giant cell arteritis

Glomus tumor

Haemangioma

Kaposi sarcoma

Leukocytoclastic vasculitis (microscopic polyarteritis)

Polyarteritis nodosa

A

Leukocytoclastic vasculitis (microscopic polyarteritis)

The patient has “palpable purpura”, caused by an allergic immune reaction to penicillin, which can be inferred by the proximity to the penicillin dose. This has caused the histologicalal finding known as leukocytoclastic vasculitis, evident in the photomicrograph. It is also known as microscopic polyarteritis, because the vessels involved are usually small, dermal vessels.

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22
Q

In the accompanying photomicrograph of a lymph node, the black arrow designates which one of the following structures?

Germinal center

Cortex

Medulla

Paracortex

Efferent lymphatic

Subcapsular sinus

A

Subcapsular sinus

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23
Q

Jim has received penicillin for pneumonias and has developed what appears to be an allergic reaction to the drug. His eye lids are puffy and his abdomen is covered with an itchy, nearly confluent hives. Wheezing in his lungs is corrected by the administration of a ß adrenergic agonist. Laboratory tests showed elevated serum histamine and decreased serum C1q and C3. Which one of the following elements of this case indicates that this reaction to penicillin was caused by circulating immune complexes, rather than an IgE-mediated allergic response?

His circulating histamine levels are elevated.

His pulmonary symptoms were corrected by giving a b-adrenergic agonist.

His C1q and C3 levels were decreased.

His skin lesions were pruritic (itchy).

The permeability of his blood vessels increased.

A

His C1q and C3 levels were decreased.

A. Elevated histamine does suggest mast cell activation, but does not indicate by what mechanism this may have occurred.
B. This class of drug works here by antagonizing the effects of histamine, but the mechanism of histamine release is not indicated by the success of this therapy.
**C. Correct. The finding of decreased C1q and C3 levels is not typical of IgE-mediated allergic reactions, but is seen in immune complex disease. It suggests that patient has produced an IgG antibody to penicillin and has undergone an "anaphylactoid" reaction to the drug. This is dependent on the production of anaphylatoxins (e.g., C3a) that activate mast cells for the release of histamine. IgE antibody is not required.**
D. Itchy skin in this patient only suggests mast cell activation and histamine release. It does not indicate the actual mechanism of mast cell activation and would not distinguish between these two mechanisms.
E. This is a typical sign associated with histamine release, but does not distinguish between several possible mechanisms of mast cell activation and histamine release.
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24
Q

Which of the following responses is LEAST LIKELY to be due to an adaptive immune response to an antigen?

Hives in an adult in response to ingested peanuts

Diarrhea in a 1-month-old child in response to infant formula

Anemia in a patient who has been repeatedly treated with penicillin

Contact dermatitis in a patient one month after changing brands of hand creams

Bronchospasms in a 12-year-old child 15 min after petting a neighbor’s dog

A

Diarrhea in a 1-month-old child in response to infant formula

All of these conditions have the appearance of allergic responses. However, one feature of allergy is the role of memory B or T cells, either as sources of IgE antibodies or memory T cells. Although childhood intolerance to formula is common, a 1-month-old child does not have the capacity to produce IgE antibodies. Thus, this disease is not due to an allergic response to an allergen.

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25
Q

An adolescent with a history of childhood upper respiratory tract infections and diarrhea continues to have recurrent sinusitis attributed to Pseudomonas aeruginosa, a gram-negative bacterium. Laboratory tests have repeatedly indicated he has normal numbers of T cells and T cell subsets, normal serum immunoglobulins, and normal neutrophil numbers and functions (chemotaxis, opsonophagocytosis, respiratory bursts). His IgG antibody response to tetanus toxoid challenge is also within the normal range and he shows strong reactions in delayed hypersensitivity skin tests to common fungal antigens. What is the appropriate therapy for this patient?

Antibiotics only

Antibiotics and IVIG

Antibiotics, IVIG and stem cell transplantation

Stem cell transplantation

Antibiotics and IFN-g

A

There is no compelling reason to think this patient has an underlying immune deficiency. Treat with antibiotics only.

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26
Q

In the accompanying high power photomicrograph of infarcted myocardium, which one of the following estimates of the age of the infarct is most likely correct?

1-2 hours

4-12 hours

1-3 days

10 days

2 weeks

1 year

A

The black arrows point to remnants of cardiac myocyte nuclei, which are undergoing karyolysis. The fibers are eosinophilic and coagulated. Neutrophils are abundant, putting the age of this infarct at about 1-3 days.

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27
Q

A 2-month-old infant is diagnosed as having a mutation in his IL-2Rγ chain gene that blocks IL-2 signaling. Which one of the following infections would be most common in this child?

Opportunistic fungal infections

Infections with gram-positive extracellular bacteria

Gram-negative bacterial pneumonias

Water-borne parasitic infections

Infections with toxin-producing bacteria, such as Clostridium tetani

A

Opportunistic fungal infections

A. Correct. Patients who lack IL-2R develop severe combined immune deficiency (SCID). Because they are protected by maternal antibodies for the first 6-8 months of life, their B cell deficiency is not clinical manifested. However, they do suffer from infections for which cell-mediated immunity is an important host defense and typically present first with opportunistic viral and fungal infections.
B. Most of host defense against these agents is mediated by antibody, complement and phagocytic cells, all of which are normal in this patient at this age.
C. Most of host defense against these agents is mediated by antibody, complement and phagocytic cells, all of which are normal in this patient at this age.
D. Although one might make the argument that defense against parasites is mediated by T cells, which would be decreased in this patient, the relative frequency of infections by other opportunistic pathogens would be much higher.
E. Host defense against toxins, most of which are proteins, is mediated by neutralizing antibodies. Antibodies to the common bacterial toxins would be expected to be passed in utero to the fetus and the child would be protected for it first half year of life by maternal antibodies.

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28
Q

Among the following, which is the most abundant subset of lymphocytes in the blood of normal healthy human beings?

CD4 T cells

CD8 T cells

B cells

Plasma cells

CD4 8 naïve T cells

A

CD4 T cells

T cells far outnumber B cells in the blood, and CD4 T cells represent approximately 70% of blood T cells. Plasma cells and naïve double positive T cells are not found in the bloodstream.

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29
Q

Circulating antigen-antibody complexes are characteristic of which one of the following sub-types of hypersensitivity?

Type I

Type II

Type III

Type IV

Type V

A

A. Type I hypersensitivity is also known as immediate type hypersensitivity, and is typically associated with “hives” and bronchoconstriction. It is due to degranulation of mast cells and release of histamine and other factors.
B. Type II hypersensitivity involves auto-antibodies against cellular or extracellular matrix components.
C. Correct. Type III hypersensitivity is due to circulating antigen-antibody complexes. Lupus is the prototypical type III disease.
D. Type IV, or delayed type hypersensitivity, is typical of the reaction seen with the PPD tuberculosis test. Macrophages must process the antigen and present it to a T cell, which takes some time to occur
E. Type V hypersensitivity does not exist.

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30
Q

A four-year-old has a defect in clearing infections caused by the polysaccharide-encapsulated bacterium Streptococcus pneumoniae. What is a possible cause for this defect?

IgA deficiency

IgG2 deficiency

IgE deficiency

MHC class II deficiency

ZAP-70 mutation

A

A. IgA deficiency is commonly asymptomatic. Those patients with IgA
B. Correct: IgG2 is a component of T-independent immune responses.
C. IgE is effective against parasitic infections.
D. Patients lacking MHC class II lack CD4+ T cells. Antibody responses against encapsulated bacteria are T-independent.
E. Patients lacking ZAP-70 have T cell defects. Antibody responses against encapsulated bacteria are T-independent.

31
Q

Which of the following laboratory tests distinguishes between a phagocytosis defect and an intracellular killing defect in neutrophils?

Nitroblue tetrazolium test

ANA test

Mixed lymphocyte response

Coombs test

Immunofixation test

A

Among the choices listed, only NBT dye reduction is a test of phagocyte function. It determines if cells can produce oxidants of the type necessary to kill microbes in phagolysosomes.

32
Q

A 5 year-old boy is referred to your clinic at the children’s hospital. He has suffered from recurrent sinus infections since about 9 months of age. When he was three, he was hospitalized with pneumonia caused by Pneumocystis carinii. All of his infections were successfully treated with antibiotics. The patient’s Ig levels are:

IgM: 210 mg/dL (75-150)
IgG: 25 mg/dL (600-1500)
IgA: undetectable (150-225)

In vitro proliferation of his B and T cells in response to polyclonal stimuli was normal. Which of the following genes is MOST likely mutated in this patient?

L-1

IL-4

IL-6

IL-12

IL-17

A

This is an unusual number and cause of infections, suggesting an immunodeficiency. High levels of IgM with very low levels of other subtypes suggests hyper-IgM syndrome. Hyper-IgM syndrome can be a B cell-intrinsic defect or a T cell defect. While CD154 is the classical mutation with hyper-IgM, any defect in the Th2 pathway would present similarly, including a lack of IL-4.

33
Q

A 4-month-old child is diagnosed as having Bare Lymphocyte Syndrome – Type II. Which of the flow cytometry patterns shown in Figure #4 correctly depicts the phenotype of the patient’s thymocytes?

A
A. This is the pattern one would see for thymocytes from a normal individual.
**B. Correct. BLS Type II is an absence of CD4+ (single positive) thymocytes due to the absence of effective positive selection on MHC class II molecules. BLS type II is due to the absence of CIITA, a transcription factor necessary for the expression of all MHC class II genes. Thus, these patients lack both class II molecules and the T cell subset restricted to recognizing antigens presented by class II MHC.**
C. This is a typical pattern for lymphocytes found in the periphery of normal individuals, but would not be encountered in the thymus. One must have double positive cells to produce single positive subsets.
D. This is the pattern of staining one might see in an athymic individual. Both double positive and single positive cells are missing.
E. This is not a possible pattern for thymocytes, because one must have double positive cells to generate the single positive CD8+ subset.
34
Q

A 2-month-old girl presents with severe lymphopenia and oral candidiasis. The clinical laboratory cultures a heavy growth of the yeast Candida albicans from a scraping of the buccal mucosum. Which one of the following is the most appropriate test to order next?

Quantitative immunolglobulins in serum

C1 Inhibitor concentrations in serum

Flow cytometry of blood lymphocytes for CD19

Anti-HIV-1 ELISA

Rheumatoid factor in serum

A

Anti-HIV-1 ELISA

Although even healthy children can get thrush, when the condition occurs in combination with lymphopenia in a very young child, one must consider a T cell immune deficiency. HIV infection being the most common, check the child’s serum for HIV antibodies.

35
Q

A 10-year-old boy has been diagnosed with chronic granulomatous disease. Which of the following host defense molecules are his cells incapable of producing?

TNF-a

IL-2

PGE2

Superoxide anion

Nitric oxide

A

CGD patients have defects in the NADPH oxidase that catalyzes reduction of oxygen to superoxide.

36
Q

An employee of a body shop presents with complaints of itchy indurated skin lesions on his forearms of 3 months duration. He has no apparent systemic symptoms. He works with paint-removing chemicals but admits he does not always wear protective clothing. You prescribe hydrocortisone cream and advise he avoid chemical exposure in the future. The lesions subside within several days. Which one of the following mechanisms of immune tissue injury MOST LIKELY contributed to his skin lesions?

Production of IgG antibodies to chemical haptens

Production of IgE antibodies to paint dust allergens

Immune complexes deposited in his epidermis

Activation of T cells and macrophages in the skin

Direct activation of the complement system by chemicals

A

Activation of T cells and macrophages in the skin

A. While IgG antibodies can be formed against haptens and might cause a rash in the skin, the indurated nature of this patient’s lesions and the response to hydrocortisone suggest a mechanism other than immune complex deposition.
B. While IgE antibodies can be formed against haptens and might cause a rash in the skin, the indurated nature of this patient’s lesions and the response to hydrocortisone suggest a mechanism other than IgE-mediated hypersensitivity.
C. While antibodies that might form immune complexes can be formed against haptens and might cause a rash in the skin, the indurated nature of this patient’s lesions and the response to hydrocortisone suggest a mechanism other than immune complex deposition.
D. Correct. The patient’s symptoms, especially the indurated lesions that subside with hydrocortisone treatment suggest a cell-mediated (type IV) reaction to the chemical hapten. This appears as a contact dermatitis and is probably delayed in its onset.
E. This mechanism is rare and would not be expected to be limited to the skine (i.e., would be expected to show systemic symptoms). Regardless, the lesions formed in the skin when complement is activated tend to be more edematous (fluid filled) and show neutrophil recruitment due to the anaphylatoxin and chemotactic activity of activated complement peptides.

37
Q

Which one of the following inciting events is most likely to give rise to granulomatous inflammation?

Chronic active hepatitis B

Peptic ulcer

Streptococcal (Pneumococcal) pneumonia

Staphylococcus skin infection

Tuberculosis

A

Tuberculosis is always associated with granulomas, except in the case of AIDS, where no CD4 cells are available.

38
Q

Two days after a group of young adults go camping, one member of the group develops an itchy, blistering rash on his ankle. The patient applies topical corticosteroids, which provide some relief. Accumulation of which of the following cells would most likely account for the lesion seen in this individual?

γδ T cells

Macrophages

Naïve CD4+ T cells

Naïve CD8+ T cells

NK cells

A

Macrophages - This is a classic picture of delayed-type hypersensitivity in response to a poisonous plant, such as poison ivy. The lesion is caused by an accumulation of macrophages and activated CD8+ T cells.

39
Q

A 12-month-old boy presents with recurrent, complicated bacterial infections and his tonsils are barely detectable. Family history reveals three healthy sisters, but an uncle died at age 10 with bacterial pneumonia. Serum Ig levels for the boy indicate low levels of Ig, despite receiving the normal childhood immunizations. Which of the following lab tests would be most appropriate and why?

Anti-HIV antibodies for suspected AIDS

Chest X-ray to determine whether the patient has a normal thymus

Flow cytometry to analyze the number of B and T cells for suspected XLA.

Flow cytometry for CD154 expression of T cells for suspected hyper-IgM syndrome

NBT dye reduction test for suspected complement deficiency

A

Flow cytometry to analyze the number of B and T cells for suspected XLA.

A. AIDS patients present with few CD4+ T cells and mostly viral infections.
B. Athymic patients would present younger and with viral infections.
C. Correct – Family history is a big clue here. Also, bacterial infections point to a B cell defect.
D. Hyper-IgM patients have normal lymphocyte counts, so the tonsils would be of normal size or larger.
E. NBT tests are not for complement deficiency

40
Q

A 19-year-old woman has met with her allergist to discuss the results of allergy skin testing and ImmunoCAP assays performed the previous week. It was noted that she had a very high ImmunoCAP test response to several antigens, but no positive skin test responses to any allergen in the panel or the histamine control. Which one of the following would explain these results in this patient?

She was taking anti-histamines at the time of the skin testing.

The ImmunoCAP test detects IgG antibodies in addition to IgE antibodies.

It is not allergy season, so her IgE antibody concentrations are low.

It is allergy season, so her IgE antibody levels are high.

She had previously undergone immunotheraphy to reduce her allergic symptoms.

A

She was taking anti-histamines at the time of the skin testing.

The absence of a positive response to histamine indicates that the patient may be taking anti-histamines. Immunotherapy would not have produced this effect.

41
Q

An enlarged mesenteric lymph node was removed from the abdomen of a 64-year-old man at autopsy, after he died in a motor vehicle accident. A representative section is shown in the accompanying photomicrograph. Which one of the following is the best diagnosis?

Diffuse large B cell lymphoma

Follicular hyperplasia

Follicular lymphoma

Hodgkin’s lymphoma

Metastatic carcinoma

A

The overall structure of the lymph node is destroyed by the structure labeled ‘T’ for Tumor. Even on low power, you can see tumor necrosis (N). This is a metastatic squamous cell carcinoma.

42
Q

What would you expect to see with a patient that has an absent C4bp?

A

The absence of C4bp would be expected to lead to stabilization of the classical and lectin pathway C3 convertases, which would depress C3 and C5 levels.

43
Q

A 44-year-old man was stung by an insect and developed hives, shortness of breath and disorientation 10-15 min later. Which of the following inflammatory mediators would be elevated in his serum 10-15 minutes after the insect sting if his symptoms are due to atopic allergic anaphylaxis?

C3b

IL-13

TNF-a

fibrinogen

histamine

A

Histamine and tryptase can be detected in the circulation of patients following acute systemic allergic responses. These are the only mediators listed that are produced in high concentrations by activated mast cells. TNF is produced by mast cells, but requires much longer periods of time than 10-15 min to be synthesized and released. Histamine is pre-synthesized and stored within mast cells.

44
Q

A 10-year-old child has a selective immune deficiency as determined by the following findings: unresponsiveness in intradermal delayed-type hypersensitivity skin testing to a panel of 8 different microbial antigens, failure of his blood T cells to secrete IL-2 in response to allogeneic cells in a mixed lymphocyte reaction and recurrent fungal and viral infections. Which of the following genetic defects would best account for this apparent immunodeficiency in this patient?

CD3 signaling defect (ZAP-70 deficiency)

Btk deficiency

Myeloperoxidase deficiency

κ locus deletion

H chain locus deletion

A

CD3 signaling defect (ZAP-70 deficiency)

This patient appears to have a selective T cell deficiency, although no specific data on T cell numbers is provided. Of the choices, only choice A (CD3 deficiency) would have produced this clinical picture. The other conditions affect B cells and phagocytes.

45
Q

A 9 year-old girl presents to your clinic with a history of recurrent bacterial infections, including several hospitalizations for bacterial pneumonia. A chest X-ray shows severe bronchiectasis. Her CBC with differential was unremarkable, but her serum immunoglobulin levels were:
IgM: 125 mg/dL (35-81)
IgG: 250 mg/dL (700-1150)
IgA: 25 mg/dL (60-110)

The gene encoding which of the following proteins is MOST LIKELY mutated in this patient?

Activation-induced cytidine deaminase (AID)

Btk

C3d

FcγRIIb

IL-5

A

A. Correct. AID, activation-induced deaminase, is required for antibody class-switching.

46
Q

A 12 year-old boy is eating dinner with his family in a restaurant when his eyes become swollen and he develops hives. Within minutes, the boy begins to have difficulty swallowing and starts to wheeze. After losing consciousness, an ambulance arrives. Which of the following drugs would be MOST APPROPRIATE as the first treatment given to the boy.

Cromolyn sodium

Dexamethasone

Epinephrine

Montelukast

Theophylline

A

A. Incorrect. Cromolyn sodium blocks mast cell degranulation prior to an allergic reaction. It is unable to reverse the anaphylactic reaction once it is started.
B. Incorrect. While corticosteroids are an essential element for sustained therapy, their effects are delayed, making them inappropriate for an emergency.
C. Correct. Epinephrine will activate the β adrenergic receptors and induce bronchodilation. This is appropriate first-line therapy.
D. Incorrect. Montelukast is a leukotriene receptor antagonist and will be unable to provide the supportive care required in an emergency.
E. Incorrect. Theophylline can block mast cell degranulation prior to an allergic reaction. It is unable to reverse the anaphylactic reaction once it is started.

47
Q

What is thrush?

A

Thrush is also known as mucocutaneous candidiasis (a yeast infection).

48
Q

In the accompanying high power photomicrograph of infarcted myocardium, which one of the following estimates of the age of the infarct is most likely correct?

1-2 hours

4-12 hours

2-3 days

10 days

2 weeks

1 year

A

The myocardium labeled ‘IN’ for Infarct, is eosinophilic, which compared to more normal myocardium (M). No cellular infiltrate is seen. Eosinophilia, due to coagulative necrosis, is one of the first histologic changes visible in infarcted myocardium, which doesn’t appear until at least 4 hours has elapsed since coronary artery occlusion. See Robbins, Table 12-5.

49
Q

In the accompanying low power photomicrograph of lung, from a patient with tuberculosis, the eosinophilic area labeled “CN” in the center of the lesion depicted is best described by which one of the following words or statements?

Apoptosis

Caseous necrosis

Cottage cheese degeneration

Liquifactive necrosis

Suppurating granuloma

A

Yes, CN stands for caseous necrosis, which is typical, but not diagnostic, of tuberculosis. The diagnosis must be confirmed by sputum culture of the mycobacteria.

50
Q

A 21-year-old woman becomes ill while eating at a local restaurant and presents to the emergency department of a local hospital with wheezing and hypotension. After having received epinephrine, antihistamines and corticosteroids, she is released with the advice to seek allergy testing to determine if she is allergic to any foods. What would be the optimal time to schedule a visit with the allergist?

Immediately (ie., the same day)

The following day

In several weeks

Only after confirming her suspected allergy by consuming a similar meal

Only after she is sure the allergic condition has disappeared

A

In several weeks

The patient should wait at least a week prior to skin testing. She has just undergone a systemic anaphylactic response, which has depleted her mast cells of allergic mediators. These cells and their mediators will need to be replenished for a skin test to show positive responses.

51
Q

In the accompanying high power photomicrograph of infarcted myocardium of about 7-10 days duration, which one of the following is a likely complication during this stage of repair?

Granuloma formation

Hemopericardium

Hypertrophic scar

Neoplastic transformation

A

Hemopericardium - The answer is B, hemopericardium, due to the fact that macrophages are digesting infarcted tissues and fibroblasts have not yet created a scar of high tensile strength. The end result is essentially dehiscence, with rupture of the LV free wall, pumping blood into the pericardium (tamponade, or hemopericardium).

52
Q

A four-year-old child has a defect in clearing infections caused by the polysaccharide-encapsulated bacterium Streptococcus pneumoniae. What is a possible cause for this defect?

IgA deficiency

IgG2 deficiency

MHC class II deficiency

ZAP-70 mutation

IgE deficiency

A

A. IgA deficiency can generally be compensated by IgM production. IgA is not produced in response to a T-independent antigen.
B. Correct. IgG2 can be generated against T-independent antigens.
C. This organism generates T-independent immune responses. MHC class II is not required.
D. This organism generates T-independent immune responses. ZAP-70 is not required.
E. IgE is not involved in T-independent immune responses.

53
Q

Which one of the following cells is the MOST CHARACTERISTIC of granulomatous inflammation?

Epithelioid histiocytes (macrophage)

Lymphocyte

Neutrophil

Langhans Giant cell

T cell

A

Epithelioid histiocytes are required to call something a granuloma. Giants cells are nice, but they are not required to make the diagnosis.

54
Q

A healthy 1-week-old male child develops a fever and is taken to a pediatric outpatient clinic. The pediatrician diagnoses acute otitis media and prescribes a course of antibiotics (oxacillin). Assuming no underlying congenital immune deficiencies, which of the following immune responses can the pediatrician assume is functional in this child at this age?

Ability to activate complement C3 deposition on microbial surfaces through the alternative pathway

Ability of dendritic cells to present microbial protein antigens to memory CD8 T cells

Ability to generate IgA-committed memory B cells

Ability of B cells to express the poly-Ig receptor

Ability to produce high-titered IgE antibodies to microbial antigens

A

Ability to activate complement C3 deposition on microbial surfaces through the alternative pathway - Complement is an innate immune system pattern recognition and effector system, which is functional in human beings at birth. Activation through the alternative pathway produces a C3 convertase, which can cleave C3 leading to deposition of the opsonin iC3b on microbial surfaces.

55
Q

A 4-month-old boy presented to your clinic with a runny dose and a persistent cough. Upon physical exam, you observed “spots” on the boy’s tongue that resembled thrush. A CBC revealed that his WBC count was 4200 cells/μL (normal 5,000-10,000). A flow cytometry panel was performed and none of the boy’s cells were bound by anti-CD3, 90% of his lymphocytes were bound by anti-CD20. None of the boy’s B cells were bound by anti-CD132 (also known as IL-2Rγ). Lack of which of the following proteins would produce a similar clinical picture as CD132 deficiency?

Btk

CD11a

IL-4

STAT5

TRAF6

A

STAT5

This patient has an immunodeficiency with a lack of T cells (CD3+cells). See the signaling pathways downstream of IL-2Rγ, also known as the common γ chain.

56
Q

A 10-year-old child falls from the roof of a garage onto a fence post and ruptures his spleen. The ruptured organ is removed and the following day his parents meet with the attending physician about recommendations for the child’s future health care needs. The child is current on all pediatric vaccines, including those for tetanus, diphtheria, pertussis, measles, mumps, rubella, hepatitis, Haemophilus and pneumococcus. When compared to the polysaccharide vaccines, what characteristic of the conjugate forms of the last two vaccines makes them particularly valuable for this child?

Greater adjuvant effects

Induction of high-titered IgG antibodies and memory B cells

Induction of cellular immunity against intracellular pathogens

Induction of mucosal immunity mediated by IgA

Lower incidence of side effects

A

A. Incorrect. There is no additional adjuvant activity associated with conjugate vaccines.
B. Correct. Compared to pure polysaccharide vaccines, protein-polysaccharide vaccines activate Th cells leading to Ig class switching to IgG (especially IgG1), a potent opsonic antibody for the extracellular Haemophilus and Pneumococcus bacterial pathogens. This is particularly important to this child, because the spleen contains B cells that would normally produce both IgM and low levels of IgG2 antibody in response to pure polysaccharide vaccines. Immunization with the conjugate vaccines assures he will also have IgG memory B cells against these pathogens.
C. Incorrect. Polysaccharide antigens are not vaccine targets for intracellular pathogens.
D. Incorrect. Conjugate vaccines are given IM, not by the oral or respiratory routes. These vaccines protect by virtue of circulating IgG antibody production, not IgA antibody induction.
E. Incorrect. There are no obvious advantages of a conjugate vaccine over a polysaccharide vaccine in this respect.

57
Q

In the accompanying high power photomicrograph of infarcted myocardium, which one of the following estimates of the age of the infarct is most likely correct?

  • 1-2 hours
  • 4-24 hours
  • 2-3 days
  • 10 days
  • 2 weeks
  • 1 year
A

2wks - Granulation tissue (“G”) has begun to appear, judging by the presence of fibroblasts, collagen fibers, and numerous small blood vessels.

58
Q

A 50-year-old male suffers from a chronic, open wound. Some tissue is removed during wound treatment (debridement). Which one of the following is the most likely diagnosis given to this tissue sample by the examining pathologist?

Angiosarcoma

Giant cell arteritis

Granulation tissue

Haemangioma

Kaposi sarcoma

Leukocytoclastic vasculitis (microscopic polyarteritis)

Polyarteritis nodosa

A

Both the history in the vignette and the finding of a highly cellular neoplasm with vascular slits in the photomicrograph should lead you to the diagnosis of Kaposi sarcoma. Modern highly active anti-retroviral therapy (HAART) has virtually eliminated the Kaposi sarcoma epidemic, which was quite sever in the 1980s in America.

59
Q

A 9-month-old child presents with pneumonia and otitis media. The laboratory cultures the bacterium Streptococcus pneumoniae from a sample of sputum, but all virus cultures for common pediatric pathogens are negative. Serum IgG levels are less than half of adult levels. Your course of action should be to:

provide antibiotics only.

provide antibiotics plus IVIG.

provide IVIG only.

provide antibiotics and IVIG and perform a skin sweat test.

provide a stem cell transplant from an HLA-matched donor.

A

A. Correct. Other than the infections, this child is normal and has no evidence of an underlying immune deficiency. The infection should be treated with antibiotics. Serum IgG levels at half the adult level are normal for a child of 9 months of age.
B. Antibiotics should be given to treat the infection, however there is no indication for IVIG. Serum IgG levels half of adult levels are normal at this age due to the decline in maternal Ig and the normal delay of neonatal Ig synthesis.
C. The infection needs to be treated with antibiotics. IVIG is not indicated, because serum IgG levels in a child of this age are normally half of adult levels.
D. Unless there is a history of recurrent pneumonia in a child, there is no indication for performing sweat tests (a test for cystic fibrosis). Antibiotics should be given to treat the infection, but IVIG should be avoided as serum IgG levels half of adult levels would be normal for a child of this age.
E. There is no indication for a stem cell transplant in this patient. Treat with antibiotics for the infection and avoid other treatments unless the patient shows evidence of an underlying immune deficiency. IgG levels that are half of adult concentrations are normal for this age.

60
Q

An Rh+ newborn presents with anemia, hepatosplenamegaly, jaundice and petechial hemorrhaging into the skin. Which of the laboratory tests, if positive, would confirm a diagnosis of erythroblastosis fetalis?

A

Coombs Test - The Coombs test will determine whether or not an anemia has an immune (or autoimmune) etiology. In this patient, it detects maternal IgG anti-RhD on the newborn’s erythrocytes

61
Q

A 25-year-old woman presents with abdominal pain of 12 h duration. She has a history of similar complaints, but has not received a diagnosis in the past. CBC is normal, but laboratory tests reveal reduced levels of C2 and C4, and the CH50 hemolytic complement test reports reduced complement function. All other complement components are normal. She has been successfully treated in the past with fresh frozen plasma, which reduced her acute symptoms. A deficiency of which of the following immune components BEST EXPLAINS these clinical findings?

C1 inhibitor

C2

C4-binding protein

Factor I

Plasmin

A

A. Correct. The presentation, test results and response to therapy are best explained by a diagnosis of hereditary angioedema, which is a deficiency of C1 Inh.

62
Q

A 54-year-old male with a long history of congestive heart failure, due to coronary artery disease brought about by smoking, presented to the Emergency Department in acute respiratory distress. He was coughing up copious amounts of pink, frothy fluid. Unfortunately, he died, and the family requested an autopsy. A representative section of his lung is shown in the accompanying photomicrograph. Which one of the following is the best diagnosis?

Adenocarcinoma of the lung

Transudate (e.g., Pulmonary edema)

Pneumonia (e.g., pneumococcus)

Pulmonary fibrosis

Pulmonary infarct

A

Transudate (e.g., Pulmonary edema)

The photomicrograph show alveoli filled with pink fluid, with very few cells. The context of the vignette tells you it is congestive heart failure, making B, transudate the correct answer. Pneumonia would be accompanied by abundant neutrophils. Infarcts in the lung are generally hemorrhagic appearing, as the bronchial arteries break down and bleed into them.

63
Q

A 23-year-old woman reports to her physician complaining of symptoms consistent with a
urinary tract infection. Having had these symptoms before, the woman tells the physician that
she has always taken Bactrim (Trimethoprim/ Sulfamethoxazole) and she felt much better. After
having her prescription filled at the local pharmacy, the woman goes to the park, takes the pill at
the drinking fountain, and joins the soccer game already in progress. Within a few minutes, she
begins to have difficulty breathing, passes out, and is taken to the emergency room.

The woman described above was treated in the emergency room and is breathing normally. The
decision is made to release the patient with instructions to see her regular physician for
follow-up care. Now that the patient is breathing normally, which of the following drugs should
she be given before leaving the hospital?

Bactrim

Celecoxib

Epinephrine

Ketoprofen

Prednisolone

A

This patient is suffering from an anaphylactoid reaction. The cause may be an allergic reaction to the Bactrim or exercise-induced asthma. The treatment is the same regardless of the cause. In either case, it is essential that a patient who experiences this severe of a response be treated with a course of steroids to prevent a secondary reaction.

64
Q

A 7-year-old child presents with itchy, erythematous, indurated lesions on her forearms and legs that first appeared two days after a camping trip with her classmates. Her mother treats her with antihistamines, which reduces the itching, but does not resolve the skin lesions. What additional therapy would probably benefit this child?

Albuterol inhaler

Topical corticorsteriods

Epinephrine

Anti-IgE

Cromolyn sodium

A

This child appears to have allergic contact dermatitis, perhaps caused by exposure to a plant resin. The delayed onset suggests a type IV hypersensitivity response, which can be effectively treated with topical hydrocortisone.

65
Q

After being treated in the emergency department for a severe asthma attack, a patient is released
with a prescription for methylprednisolone. The patient is instructed to take the steroid on the
following schedule:

1st day: 2 tablets before breakfast, 1 tablet after lunch and after supper, and 2 tablets at bedtime.
2nd day: 1 tablet before breakfast. 1 tablet after lunch and after supper, and 2 tablets at bedtime.
3rd day: 1 tablet before breakfast, after lunch, after supper and at bedtime.
4th day: 1 tablet before breakfast, after lunch and at bedtime.
5th day: 1 tablet before breakfast and at bedtime.
6th day: 1 tablet before breakfast.

Which of the following mediators necessitates the use of steroids for six days after the patient is
released from the emergency department?

ECF-A

Histamine

Serotonin

Tryptase

Tumor necrosis factor α

A

Tumor necrosis factor α

This steroid regimen is from the instructions on the Medrol DosePak, which is common given to patients in this situation. The goal is to prevent the transcription-dependent mediators from initiating a secondary reaction. Even though the secondary reaction may involve all these mediators, the goal is to block the transcriptional events.

66
Q

Decreased levels of plasmin would have what result?

A

Decreased levels of plasmin would relate more to coagulopathies. Its influence on complement activation is through cleavage of C3, so one would not expect consumption of C3.

67
Q

Which of the following effects of epinephrine explains its value in treating acute allergic anaphylaxis?

Vasodilation

Bronchodilation

Inhibition of IgE synthesis

Inhibition of cytokine gene transcription

Inhibiton of cyclooxygenase activity

A

Bronchodilation

Epinephrine stimulates cells through b-adrenergic receptors and reverses many of the effects of histamine. Histamine causes vasodilation and bronchoconstriction.

68
Q

An elderly man in long-term assisted living develops a urinary tract infection and admitted to a neighboring hospital. He is given the antibiotics ampicillin and ceftriaxone IV, both of which he has received several times in the past. Three hours later, he develops severe hypotension, tachycardia and tachypnea and loses consciousness. With appropriate care, he recovers and is skin tested with the two antibiotics several weeks later. The skin tests are negative. Which one of the following immune mediators would BEST EXPLAIN his acute reaction to these drugs?

C5b

Cytotoxic T cells

IgG antibodies

IL-5

NK cells

A

IgG Antibodies - IgG antibodies to drugs and other small molecules can cause a systemic inflammatory response syndrome (SIRS) by binding to the antigen in circulation, activating complement and liberating anaphylatoxins from the complement system. We call this rapid response to antigen challenge an “anaphylactoid” reaction only because it does not involve IgE antibodies. Also called “serum sickness” when the antigen is a large protein.

69
Q

The woman has had three earlier pregnancies with the same spouse and has not received Rhogam at any time. What is the most likely reason that this pregnancy did not result in Rh disease of the most recent newborn?

The mother was immunosuppressed by her pregnancy.

There are no incompatibilities between mother and this child.

This fetus had made antibodies to the mother’s erythrocytes.

The mother’s isohemaaglutinins cleared the fetal erythrocytes from previous pregnancies.

The mother has a deficiency in one of her complement components.

A

The mother’s isohemaaglutinins cleared the fetal erythrocytes from previous pregnancies. - Induction of anti-Rh antibodies in an Rh- mother by Rh+ fetal erythrocytes requires that the fetal cells survive in the mother’s circulation and travel to lymphoid tissues to induce isotype switching in B cells. If the mother is A, she has preformed isohemagglutinins against the B erythrocyte antigen. The child’s erythrocytes are B and are susceptible to clearance by mother’s isohemagglutinins. This means the fetal cells are not available to induce a maternal antibody response.

70
Q

Jesse is a 10-year-old child who complains of chest tightness and wheezing after gym class and upon exposure to cold outside air. He experiences sneezing, nasal itching and nasal congestion in doors and following contact with his friend’s dog. His allergist has determined by skin testing that he is allergic to oak pollen, Bermuda grass, host dust and dog dander. His pulmonary function tests indicate an FEV1 <60% of the expected value for his age and a PFR of 180 L/min (normal >350 L/min). Which of the following drugs would be MOST BENEFICIAL in alleviating Jesse’s pulmonary symptoms?

Albuterol

Anti-TNF- α monoclonal antibody

IVIG

Cyclosporin

Recombinant IL-4

A

Albuterol

A. Correct. Albuterol is the drug of choice in this case of allergic asthma. It acts by relaxing bronchial smooth muscle and lessening bronchial constriction.
B. There is no convincing evidence that TNF- is responsible for the symptoms of asthma, and administering it might even put the patient at risk for infection.
C. Intravenous immunoglobulin is not known to be beneficial in asthma.
D. The use of such a powerful immunosuppressive drug for the treatment of asthma is not indicated. Although it may lessen Th cell activation and IgE synthesis, its side effects are too significant to indicate its use here.
E. IL-4 would be expected to increase the severity of the patient’s symptoms as it plays a role in the pathogenesis of the underlying allergic response (Th4 activation, IgE switching and B cell activation) as well as eosinophil recruitment.

71
Q

What does Cromolyn sodium do?

A

Blocks mast cell degranulation prior to an allergic reaction. It is unable to affect an allergic reaction once it has started

72
Q

A one-month-old infant with recurrent viral infections shows lymphopenia but normal serum IgG levels. His T and B cell counts are shown below.

Among the following, which is the MOST LIKELY diagnosis?

X-linked agammaglobulinemia

Chediak Higashi syndrome

Leukocyte adhesion defect

RAG-1 deficiency

MHC class I deficiency

A

RAG-1 deficiency

This patient shows significant decreases in the number of B and T cells. Among the choices, only Rag-1 deficiency would produce this feature. XLA and MHC class I deficiency result in B cell and T cell subset deficiencies, respectively.

73
Q

You have been following an 8-month-old child with an apparent antibody deficiency and have narrowed your diagnosis to two possibilities. Which one of the following tests would allow you to distinguish between hyper IgM syndrome and X-linked agammaglobulinemia?

Measurement of IgG levels in the serum

Measurement of CD19+ cells in the blood

Measurement of IL-2 production by the patient’s lymphocytes in vitro

Measurement of NK cell-mediated cytotoxicity in vitro

Measurement of IgA levels in saliva

A

A. Serum IgG concentrations would be decreased in both conditions and, at this age, would reflect mostly maternally-derived IgG
B. Correct. XLA is the only human antibody deficiency that reflects the absence of B cells (i.e., CD19+ cells) in the periphery. This is because XLA is a deficiency of Btk, a tyrosine kinase necessary for signaling a critical developmental progression in bone marrow pre-B cells.
C. IL-2 production in vitro is primarily a measurement of T cell activation and reflects activation of the Th1 subset. Even in hyper IgM syndrome, where Ig isotype switching is defective, the production of B cell switch cytokines is normal.
D. NK cells are not known to affect B cell development directly and should be normal in both of these conditions.
E. Salivary IgA levels should be depressed or absent in both conditions.