Immuno Set 8 Flashcards
Which of the following is typically NOT associated with T cell immunodeficiencies?
A. Cancer
B. Viral infections
C. Autoimmunity
D. Infection with intracellular microbes
E. Infections with pyogenic bacteria
E. Infections with pyogenic bacteria
In which of the following conditions would you be LEAST likely to see a robust delayed-type hypersensitivity (DTH) response to a Candida antigen skin challenge?
A. X-linked agammaglobulinemia
B. DiGeorge syndrome
C. Selective IgA deficiency
D. Common variable immunodeficiency
E. Chronic granulomatous disease
B. DiGeorge syndrome
A 7-month-old boy is evaluated in the clinic for recurrent bacterial infections. His tonsils are not visible of physical examination. Serum IgA and IgG levels are markedly decreased and serum IgM levels are slightly above normal. Patient’s presentation is consistent with which of the following disorder?
A. X-linked agammaglobulinemia
B. X-linked severe combined immunodeficiency disease
C. Common variable immunodeficiency
D. X-linked hyper-IgM syndrome
E. ADA deficiency
D. X-linked hyper-IgM syndrome
A 6-month-old infant presents with oral candidiasis (thrush). Laboratory blood analysis revealed normal numbers of B cells and very few CD3+ cells. In addition, maternally-derived T cells, as determined by HLA typing, are found. The infant’s mother reports that two of her brothers died as young children. This presentation consistent with which of the following of infection disorder?
A. X-linked agammaglobulinemia
B. X-linked severe combined immunodeficiency disease
C. Common variable immunodeficiency
D. X-linked hyper-IgM syndrome
E. ADA deficiency
B. X-linked severe combined immunodeficiency disease
A 6-month-old infant presents with oral candidiasis (thrush). Laboratory blood analysis revealed normal numbers of B cells and very few CD3+ cells. In addition, maternally-derived T cells, as determined by HLA typing, are found. The infant’s mother reports that two of her brothers died as young children.
Based on the family’s history and clinical presentation above, a mutation in which of the following genes is most likely to be found in this patient?
A. Jak3 kinase
B. Adenosine deaminase
C. Common cytokine receptor y chain
D. RAG2
E. Purine nucleoside phosphatase
C. Common cytokine receptor y chain
A 6-month-old infant presents with oral candidiasis (thrush). Laboratory blood analysis revealed normal numbers of B cells and very few CD3+ cells. In addition, maternally-derived T cells, as determined by HLA typing, are found. The infant’s mother reports that two of her brothers died as young children.
This infant should NOT be given the measles-mumps-rubella (MMR) vaccination because which of the following?
A. The vaccine would have no effect because he is not susceptible to infection with measles, mumps, or rubella.
B. The vaccine would have no effect because he cannot mount an appropriate humoral response.
C. The vaccine could not be dangerous because he is predisposed to development of allergic reactions to the MMR vaccine.
D. The vaccine could be dangerous because vaccination with MMR would put him as risk for developing a fatal infection.
E. Vaccination with MMR is not contraindicated in his case and should be performed to prevent future infections.
D. The vaccine could be dangerous because vaccination with MMR would put him as risk for developing a fatal infection.
A lymph node biopsy from a young boy with X-linked agammaglobulinemia is expected to show which of the following?
A. Marked perivascular lymphocytic infiltrate
B. Abundant polymorphonuclear infiltration
C. Reduced follicles and germinal centers
D. Nearly absent parafollicular cortical regions
E. Enlarged follicles
C. Reduced follicles and germinal centers
Which of the following is the most common known primary immunodeficiency with a prevalence of 1 in 700 white individuals?
A. X-linked agammaglobulinemia
B. Selective IgA deficiency
C. Common variable immunodeficiency
D. Chronic granulomatous disease
E. Chediak-Higashi syndrome
B. Selective IgA deficiency
An 18-month-old female presents with recurrent respiratory tract infections. She has a fever, labored breathing, and a dry cough. Laboratory investigations confirm infection with Pneumocystis jiroveci. The family history is unremarkable; the patient’s three older brothers are in excellent health. This patient most likely carries a mutation in which of the following molecules?
A. CD18
B. CD40L
C. ZAP-70
D. CD80
E. CD40
E. CD40
A 3-year-old male presents with a nosebleed. The past medical history is remarkable for severe recurrent sinopulmonary infections. Physical examination revealed eczema and multiple tiny hemorrhagic spots. Laboratory blood findings include thrombocytopenia and reduced IgM levels. The boy’s maternal uncle died of bleeding complications after an emergency appendectomy. Which of the following is the most likely diagnosis?
A. X-linked hyper IgM syndrome
B. Wiskott-Aldrich syndrome (WAS)
C. Chediak-Higashi syndrome
D. Transient hypogammaglobulinemia of infancy
E. DiGeorge syndrome
B. Wiskott-Aldrich syndrome (WAS)
A 2-month-old male infant presents with persistent diarrhea, signs and symptoms of Pneumocystis carinii pneumonia, and an oral fungal infection with Candida albicans. His weight is in the 10th percentile. Test results for HIV are negative by polymerase chain reaction. Which of the following is the most consistent with findings in this patient?
A. grossly reduced levels of B cells
B. an X-linked inheritance of HLA genes
C. defective isotype switching
D. defective T-cell function
E. selective lgA deficiency
D. defective T-cell function
A 5-year-old girl has a small deletion in chromosome 22. She has impaired thymus development with a significant deficiency in the number of functional T cells. Which of the following is the most likely etiology of this patient clinical presentation?
A. Adenosine deaminase (ADA) deficiency
B. Chediak-Higashi syndrome
C. DiGeorge syndrome
D. Hereditary angioedema
E. Severe combined immunodeficiency (SCID)
C. DiGeorge syndrome
A 3-year-old boy with an X-linked defect in the Bruton tyrosine kinase (BTK) gene is impaired in which of the following mechanisms?
A. Antibody-mediated bacterial clearance
B. Formation of the membrane attack complex
C. Inability to produce respiratory burst
D. IFN-y secretion by CD4 + T cells
E. T-cell precursor migration to the thymus
A. Antibody-mediated bacterial clearance
A 6-month-old male infant has diarrhea, extensive fungal infections, and skin rashes and has failed to gain weight. He is deficient in both T- and B-cell function. The thymus is of normal size. Which of the following would be the most likely therapy for permanent restoration of immunity in this patient?
A. an antibiotic “cocktail” given at regular intervals
B. bone marrow transplantation
C. exogenous immunoglobulins administered periodically
D. isolation to an antiseptic environment
E. thymic hormones given throughout his life
B. bone marrow transplantation
A female neonate has cardiac abnormalities and hypocalcemia in addition to the diminished cell- mediated and B-cell responses. Which of the following immune deficiencies should be included in the differential diagnosis of this patient?
A. Adenosine deaminase (ADA) deficiency
B. DiGeorge syndrome
C. Hereditary angioedema
D. Severe combined immunodeficiency disease (SCID)
E. Wiskott-Aldrich syndrome
B. DiGeorge syndrome
A 21 -year-old woman has a history since childhood of recurrent episodes of swelling of the submucosal and subcutaneous tissue of the gastrointestinal and respiratory tracts. Her C1 inhibitor level is less than 5% of the reference value. These findings support a diagnosis of which of the following?
A. DiGeorge syndrome
B. Hereditary angioedema
C. Malnutrition-based immunodeficiency
D. Paroxysmal nocturnal hemoglobinuria
E. Wiskott-Aldrich syndrome
B. Hereditary angioedema
A 3-month-old male infant has recurrent infections and is found to have an impaired ability to kill microbes by the nitroblue tetrazolium test (which evaluates effectiveness of oxidase enzymes). Which of the following conditions is most likely responsible for the findings in this patient?
A. Chediak-Higashi syndrome
B. Chronic granulomatous disease
C. Hereditary angioedema
D. Common Variable Immune Deficiency
E. Common y chain deficiency
B. Chronic granulomatous disease
A 4-year-old child is referred with the diagnosis of type 1 LAD. She has a history of recurrent bacterial infections, including pneumonias and skin abscesses. Radiography demonstrates granuloma-like lesions in her lungs and liver. However, the data in the following table suggest this condition has not been properly diagnosed. Which of the elements in the table is inconsistent with a diagnosis of type 1 LAD?
A. The CD2 value
B. The CD19 value
C. The CD11a value
D. The CD45 value
E. The CD3 value
C. The CD11a value
A 3-month-old child presents with a history of persistent diarrhea and oral thrush beginning at age 3 weeks and has developed signs and symptoms consistent with Pneumocystis carinii pneumonia. Both he and his parents are HIV negative based on serological and polymerase chain reaction (PCR) testing. Complete blood counts demonstrate profound lymphopenia. Which of the following blood cell types should show normal numbers if this condition was caused by a Rag deficiency?
A. CD3+ cells with a CD4 coreceptor
B. CD19+ cells
C. Single-positive thymocytes
D. Memory B cells
E. NK cells
E. NK cells
Measuring which of the following immune parameters would distinguish hyper-IgM syndrome from X-linked agammaglobulinemia?
A. IgG levels in the serum
B. CD19+ cells in the blood
C. IL-2 production by the patient’s lymphocytes in vitro
D. NK cell-mediated cytotoxicity in vitro
E. IgA levels in the saliva
B. CD19+ cells in the blood
A 2-year-old boy is evaluated for a severe combined immunodeficiency disease. His bone marrow has normal cellularity. Radioactive tracer studies demonstrate a normal number of T-cell precursors entering the thymus, but no mature T lymphocytes are found in the blood or peripheral organs. Cells populating the thymus are found to lack CD3. Which of the following capabilities would his cells lack?
A. Ability to bind cell-bound peptides
B. Ability to express CD4/CD8 coreceptors
C. Ability to produce terminal deoxynucleotidyl transferase
D. Ability to proliferate in response to specific antigen
E. Ability to rearrange T-cell receptor gene segments
D. Ability to proliferate in response to specific antigen
A young woman with acute myeloblastic leukemia is treated with intensive chemotherapy and achieves remission of her symptoms. Because the prognosis for relapse is relatively high, a bone marrow transplant is undertaken in her first remission. Which of the following cytokines administered with the bone marrow cells would have the beneficial result of stimulating lymphoid-cell development from the grafted stem cells?
A. IL-1
B. IL-2
C. IL-3
D. IL-6
E. IL-7
E. IL-7
A lymph node biopsy of a 6-year-old boy shows markedly decreased numbers of lymphocytes in the paracortical areas. Analysis of his peripheral blood leukocytes is likely to show normal to elevated numbers of cells expressing which of the following surface marker?
A. CD2
B. CD3
C. CD4
D. CD8
E. CD19
E. CD19
An 8-month-old baby has a history of repeated Gram-positive bacterial infections. Which of the following is most probable cause for this condition?
A. the mother did not confer sufficient immunity to the baby in utero
B. the baby suffers from erythroblastosis fetalis (hemolytic disease of the newborn)
C. the baby has a defect in the alternative complement pathway
D. the baby is allergic to the mother’s milk
E. none of the above
E. none of the above
