Chapter 06: Diseases of the Immune System
Test Bank
MULTIPLE CHOICE
- Which of the following is the most abundant bronchospasm-inducing arachidonic acid derivative generated by cyclooxygenase in the mast cells?
| a. | Leukotriene B4 |
| b. | Prostacyclin |
| c. | Prostaglandin D2 |
| d. | Thromboxane |
| e. | Lipoxin |
ANS: C, Prostaglandin D2 is the most abundant arachidonic acid derivative produced through the cyclooxygenase pathway. It causes bronchospasm and increased mucus production.
- A 40-year-old man presents with hemoptysis and renal failure. A kidney biopsy specimen revealed linear deposits of IgG in glomerular basement membranes and crescents between the glomerular capillary loops and the Bowman capsule. The antibodies causing these pathologic changes react with
| a. | laminin |
| b. | fibronectin |
| c. | collagen type I |
| d. | collagen type IV |
| e. | podocytes of glomerular epithelial cells |
ANS: D, This man has Goodpasture syndrome, in which the body produces autoantibodies to the globular part of type IV collagen in the glomerular basement membranes and the lungs.
- Which antibody can most effectively activate complement by the alternate pathway, even without prior forming an antigen-antibody complex?
| a. | IgA |
| b. | IgD |
| c. | IgE |
| d. | IgG |
| e. | IgM |
ANS: A, IgA can activate complement by the alternate pathway.
- Antigen-antibody reaction in polyarteritis nodosa is characterized by which form of necrosis of the arterial wall?
| a. | Coagulative necrosis |
| b. | Fibrinoid necrosis |
| c. | Liquefactive necrosis |
| d. | Fat necrosis |
| e. | Caseous necrosis |
ANS: B, Antigen-antibody complexes cause inflammation of the vessel wall, accompanied by the insudation of plasma proteins into the site of injury. The necrosis is classified as fibrinoid, because it contains large amounts of fibrin formed from fibrinogen in the plasma insudated into the damaged vessel wall.
- A 23-year-old woman was given an intradermal injection of purified protein derivative of tubercle bacilli (PPD) as part of the yearly testing for tuberculosis. A positive reaction was elicited. Which cells secrete the cytokines needed for the formation of granulomas at the site of injection?
| a. | Keratinocytes |
| b. | Melanocytes |
| c. | B lymphocytes |
| d. | TH1 cells |
| e. | TH2 cells |
ANS: D, Granuloma formation depends on cytokines produced by TH1 cells derived from CD4+ lymphocytes.
- The formation of vesicles in contact dermatitis caused by poison ivy is primarily mediated by
| a. | TH1 lymphocytes |
| b. | TH2 lymphocytes |
| c. | CD8 lymphocytes |
| d. | B cells |
| e. | macrophages |
ANS: A, TH1 helper lymphocytes accumulate first at the site of exposure to the foreign antigen in the skin; they also are the most important mediators of epidermal cell injury that leads to formation of vesicles and bullae. CD8 cytotoxic cells also take part in later stages of the reaction, but to a lesser degree.
- Generic antinuclear antibodies are found in more than 95% of patients with systemic lupus erythematosus. Which pattern of staining seen on tissue culture cells in the indirect antibody immunofluorescence microscopy test signifies the presence of these antibodies?
| a. | Nucleolar pattern |
| b. | Speckled nuclear pattern |
| c. | Diffuse nuclear pattern |
| d. | Rim nuclear pattern |
| e. | Granular cytoplasmic pattern |
ANS: C, Generic antinuclear antibodies (ANA) reacting with chromatin, histones, and occasionally double-stranded DNA give a diffuse nuclear staining pattern. Rim (or peripheral) staining is due to antibodies to double-stranded DNA. A speckled pattern is typical of antibodies to non-DNA nuclear components, including the Sm-antigen, which is the most specific marker of systemic lupus erythematosus. A nucleolar pattern reflects the presence of antibodies to nucleolar RNA. Cytoplasmic staining is nonspecific and unrelated to antinuclear antibodies.
- The most common and the most serious form of renal disease encountered in systemic lupus erythematosus (SLE) is classified as x. Most of the lesions found in SLE are mediated by the deposition of immune complexes (type III hypersensitivity reaction). Which pathologic reaction is due to type II hypersensitivity (cytotoxic) reaction?
| a. | Class I (minimal changes) |
| b. | Class II (mesangial) |
| c. | Class III (focal proliferative) |
| d. | Class IV (diffuse proliferative) |
| e. | Class IV (membranous) |
ANS: D, The most common and clinically the most serious form of lupus nephritis is class IV, diffuse proliferative glomerulonephritis.
- Which of the following is the predominant and most common cardiovascular manifestation of systemic lupus erythematosus?
| a. | Mural endocarditis |
| b. | Valvular endocarditis of mitral valve |
| c. | Myocarditis |
| d. | Pericarditis |
| e. | Conduction system injury with arrhythmia |
ANS: D, Pericarditis is the most common cardiovascular manifestation of systemic lupus erythematosus.
- Which antibodies are considered to be markers of Sjögren syndrome?
| a. | Antinuclear antibody |
| b. | Anti-double-stranded DNA |
| c. | Anti-single-stranded DNA |
| d. | Anti-topoisomerase |
| e. | Anti-ribonucleoprotein antibody SS-A (Ro) |
ANS: E, Anti-ribonucleoprotein antibodies (RNP) are found in about 90% patients with Sjögren syndrome and are found in less than 5% of patients with other autoimmune diseases. Hence, these anti-RNP antibodies, called SS-A and SS-B, are very good markers for this disease.
- Which of the following blood studies is the best evidence of microvascular endothelial cell injury that typically occurs in systemic sclerosis (scleroderma)?
| a. | Hyperbilirubinemia |
| b. | Hypoalbuminemia |
| c. | Increased levels of von Willebrand factor |
| d. | Increased levels of IgG |
| e. | Increased levels of fibrinogen |
ANS: C, Von Willebrand factor is produced and stored in endothelial cells. An endothelial cell injury leads to elevated levels of this factor in blood.
- Antibodies to centromere are found in patients who have
| a. | systemic sclerosis |
| b. | CREST syndrome |
| c. | Sjögren syndrome |
| d. | polymyositis |
| e. | dermatomyositis |
ANS: B, Almost all patients who have anti-centromere antibodies have a form of scleroderma called CREST. Symptoms of CREST syndrome, a limited form of scleroderma, are calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, and telangiectasis. In contrast to CREST syndrome, systemic sclerosis is characterized by the appearance of antibodies to DNA topoisomerase (anti-Scl 70).
- A 10-year-old girl presented with lilac or heliotrope discoloration of the upper eyelids, muscle weakness, and dusky red patches over the knuckles, elbows, and knees. Which antibody was most likely found in this patient’s blood?
| a. | Anti-histidyl-t-RNA synthetase |
| b. | Anti-double-stranded DNA |
| c. | Anti-single-stranded DNA |
| d. | Anti-topoisomerase |
| e. | Anti-ribonucleoprotein antibody SS-A (Ro) |
ANS: A, This girl most likely has dermatomyositis. The antibody most commonly found in inflammatory myopathies is directed against the histidyl-t-RNA.
- Mutation of the gene encoding the enzyme that is essential for the maturation of pre-B cells to mature B lymphocytes is the cause of
| a. | common variable immunodeficiency |
| b. | isolated IgA deficiency |
| c. | DiGeorge syndrome |
| d. | severe combined immunodeficiency |
| e. | X-linked agammaglobulinemia |
ANS: E, X-linked agammaglobulinemia (Bruton agammaglobulinemia) is caused by the mutation of Bruton tyrosine kinase (Btk), an enzyme essential for the maturation of pre-B cells into B lymphocytes. Because the defective B lymphocytes cannot produce immunoglobulins, there is agammaglobulinemia and reduced resistance to infections.
- Autoantibodies to IgA are found in approximately 40% of patients who have
| a. | common variable immunodeficiency |
| b. | isolated IgA deficiency |
| c. | DiGeorge syndrome |
| d. | severe combined immunodeficiency |
| e. | X-linked agammaglobulinemia |
ANS: B, Although they lack IgA in their blood and secretion, patients with congenital isolated IgA often have antibodies to IgA. Transfusion of blood to these patients may result in severe anaphylactic reaction due to the reaction of the patient’s autoantibodies and the IgA in the transfused blood.
- Which molecule on the surface of lymphocytes serves as the receptor for the human immunodeficiency virus (HIV)?
| a. | CD3 |
| b. | CD4 |
| c. | CD8 |
| d. | CD15 |
| e. | CD20 |
ANS: B, CD4, the cell surface marker of T-helper lymphocytes, serves as a high affinity receptor for the human immunodeficiency virus.
- In addition to T-helper lymphocytes, which other cells serve as a major reservoir for the human immunodeficiency virus in lymph nodes?
| a. | B lymphocytes |
| b. | Pre-B and pre-T lymphocytes |
| c. | Cytotoxic T lymphocytes |
| d. | Follicular dendritic cells |
| e. | Plasma cells |
ANS: D, The human immunodeficiency virus lives and replicates in CD4+ T-helper lymphocytes and follicular dendritic cells in the germinal centers of lymph nodes.
- The most common fungal pathogen infecting the mouth and the esophagus of patients with AIDS is
| a. | Candida albicans |
| b. | Cryptococcus neoformans |
| c. | Aspergillus fumigatus |
| d. | Histoplasma capsulatum |
| e. | Cryptosporidium |
ANS: A, In general, the most common fungal pathogens in patients with AIDS is Candida albicans. Most often, it infects the mouth and the esophagus, but it can be found in many internal organs such as the vagina.
- Amyloid deposited in the tissue of patients who are on dialysis for chronic renal failure is most often biochemically related to which precursor protein?
| a. | Light chain of immunoglobulin G |
| b. | Serum amyloid associated (SAA) protein |
| c. | Transthyretin |
| d. | ß2-microglobulin |
| e. | Calcitonin |
ANS: D, ß2-microglobulin is a component of the major histocompatibility complex on the cell surface and is also normally present in deposits in the tissues of patients on long-term hemodialysis, forming amyloid fibrils.
- Deposition of AL amyloid in the kidneys of patients with multiple myeloma usually results in
| a. | acute renal failure |
| b. | tubular necrosis |
| c. | nephrotic syndrome |
| d. | chronic pyelonephritis |
| e. | nephrocalcinosis |
ANS: C, Deposits of AL amyloid are typically found in the glomeruli, causing proteinuria. Proteinuria is often severe, leading to a full blown nephrotic syndrome.
Kumar: Robbins and Cotran Pathologic Basis of Disease, 8th Edition
Chapter 07: Neoplasia
Test Bank
MULTIPLE CHOICE
- Which of the following tumors occurs most often in the ovary?
| a. | Chondroma |
| b. | Hemangioma |
| c. | Leiomyoma |
| d. | Transitional cell papilloma |
| e. | Cystadenoma |
ANS: E, Cystadenomas, which may contain serous fluid or mucin and are thus called serous or mucinous cystadenomas, are typically found in the ovary. These benign tumors have malignant counterparts known as cystadenocarcinomas.
- Which one of the following carcinomas is locally invasive but rarely forms metastases in lymph nodes and distant sites?
| a. | Basal cell carcinoma of the skin |
| b. | Squamous cell carcinoma of the skin |
| c. | Oat cell carcinoma of the lung |
| d. | Transitional cell carcinoma of the urinary bladder |
| e. | Adenocarcinoma of the stomach |
ANS: A, Basal cell carcinomas of the skin, the most common malignant tumor in the human body, is a locally invasive malignant tumor, which, if untreated, could ultimately kill the host. However, these tumors are usually diagnosed early and removed adequately, so that they almost never metastasize.
- Pseudomyxoma peritonei is a complication of which tumor?
| a. | Serous cystadenoma of the ovary |
| b. | Mucinous cystadenoma of the ovary |
| c. | Ovarian fibroma |
| d. | Myxoma of the heart |
| e. | Myxosarcoma of the lower extremity |
ANS: B, Pseudomyxoma of the ovary usually results from peritoneal seeding of mucin-secreting tumors of the ovary or the gastrointestinal tract, and most often the appendix. Although the ovarian tumor is benign, the implants of the tumor cells seeding the peritoneal cavity can be difficult to eradicate. The cells continue to secrete mucin and fill the abdominal cavity, colloquially known as “jelly-belly.”
- Which mechanism affecting the genes or chromosomes accounts for the activation of the c-myc protooncogene in Burkitt lymphoma cells?
| a. | Overexpression |
| b. | Amplification |
| c. | Point mutation |
| d. | Translocation |
| e. | Inactivation of its promotor |
ANS: D, Translocation of the c-myc protooncogene from its normal site on chromosome 8 to chromosome 14 results in the formation of the myc oncogene and plays a major role in the pathogenesis of Burkitt lymphoma.
- Homogeneous staining regions (HSR) of chromosome 2p, the site of the N-myc gene, are adverse prognostic signs if found in neuroblastoma cells. HRS are evidence of gene
| a. | deletion |
| b. | amplification |
| c. | point mutation |
| d. | translocation |
| e. | inversion |
ANS: B, Homogeneous staining regions and double minutes are signs of gene amplification. Amplification of the N-myc oncogene in neuroblastoma cells is a poor prognostic finding.
- In which cell function is BRCA1, a gene mutated in women with familial breast carcinoma, involved?
| a. | Cell adhesion |
| b. | Growth inhibition |
| c. | Signal transduction |
| d. | Cell surface channel |
| e. | Transcriptional regulation and DNA repair |
ANS: E, BRCA1 and BRCA2 are tumor suppressor genes whose true function has not been fully elucidated. It is thought that they participate in the transcriptional regulation and interact with proteins that regulate double-strand DNA repair.
- Germ line mutation of E-cadherin is found in familial cases of
| a. | lymphoblastic lymphoma |
| b. | Ewing sarcoma |
| c. | neuroblastoma |
| d. | gastric carcinoma |
| e. | seminoma |
ANS: D, E-cadherin is a cell-to-cell adhesion molecule that serves to hold many epithelial cells together. This surface adhesion molecule is often lost in adenocarcinomas, accounting for the invasive growth of these neoplasms. Germ line mutation of the E-cadherin gene has been found in families with gastric carcinoma.
- Germ line mutation of the Von Hippel–Lindau (VHL) gene is associated with formation of retinal angiomas and
| a. | glioblastoma multiforme |
| b. | oligodendroglioma |
| c. | neurinomas |
| d. | ependymomas |
| e. | cerebellar hemangioblastomas |
ANS: E, Von Hippel–Lindau syndrome was originally described as a syndrome comprising angiomas of the retina and hemangioblastomas of the cerebellum. It is linked to the germ line mutation of the VHL tumor suppressor gene. These patients are at risk of developing renal cell carcinoma and pheochromocytomas. Mutations of the VHL gene also have been found in sporadic renal carcinomas unrelated to Von Hippel–Lindau syndrome.
- Microsatellite instability, a marker of DNA mismatch repair, is typically found in
| a. | hereditary nonpolyposis colon cancer |
| b. | familial adenomatous polyposis coli |
| c. | T-cell lymphoma |
| d. | B-cell lymphoma |
| e. | myelogenous leukemia |
ANS: A, Mutations of human DNA mismatch repair genes play a pathogenetic role in the development of colonic cancers in families with hereditary nonpolyposis colon cancer (HNPCC). Due to the error in mismatch repair, the microsatellite sequences (normally fixed for life and the same in every tissue) expand. They account for the microsatellite instability, a hallmark of all conditions with defective DNA mismatch repair.
- Matrix metalloproteinases secreted by tumor cells are important for which feature of malignant tumors?
| a. | Desmoplasia |
| b. | Anaplasia |
| c. | Invasiveness |
| d. | Apoptosis |
| e. | Differentiation |
ANS: C, Matrix metalloproteinases, such as type IV collagenase, are important for the invasive growth of tumor cells. These enzymes degrade the basement membranes and the extracellular matrix, allowing the tumor cells to penetrate into the tissues, lymphatics, and blood vessels.
- Which enzymes in the liver account for the activation of most procarcinogens into carcinogens?
| a. | Cytochrome P-450-dependent mono-oxidases |
| b. | Aminotransferases |
| c. | Hydroxylases |
| d. | Glutathione dehydrogenase |
| e. | Caspases |
ANS: A, Most of the exogenous procarcinogens are activated into carcinogens in the liver through the action of microsomal cytochrome P-450–dependent mono-oxidases found in the smooth endoplasmic reticulum of hepatocytes. Susceptibility to carcinogens is in part determined by the polymorphism of genes that encode these enzymes.
- Which is the most common radiation-induced cancer in humans?
| a. | Thyroid cancer |
| b. | Chronic lymphocytic leukemia |
| c. | Breast cancer |
| d. | Lung cancer |
| e. | Salivary gland carcinoma |
ANS: B, Leukemias represent the most common radiation-induced cancer in humans, closely followed by thyroid cancer, which is the most common malignancy in young persons.
- Helicobacter pylori infection predisposes to formation of gastric tumors, but the association between the bacterial infection and a gastric tumor is the strongest for
| a. | adenocarcinoma of the stomach |
| b. | gastrointestinal stromal tumors |
| c. | marginal zone lymphomas |
| d. | T-cell lymphoma |
| e. | Burkitt lymphoma |
ANS: C, The best and strongest evidence links Helicobacter pylori infection with the onset of mucosa-associated B-cell lymphomas (MALTomas) of the stomach, which are also known as marginal zone lymphomas. It is thought that H. pylori activates T cells, which in turn promote polyclonal proliferation of B cells in the gastric mucosa. In this process, some cells obviously become malignant and give rise to T-cell independent low-grade monoclonal lymphomas.
- Clubbing of the digits, periosteal new bone formation on the metatarsals, metacarpals, and proximal phalanges, and arthritis of adjacent joints is a paraneoplastic syndrome most often associated with carcinoma of the
| a. | thyroid |
| b. | larynx |
| c. | bronchi |
| d. | liver |
| e. | stomach |
ANS: C, Clubbing of the fingers and related bone and joint changes of the hand and fingers are signs of hypertrophic osteoarthropathy, which most often occurs in patients with bronchogenic carcinoma. The pathogenesis of these changes is not known.
