DIRECTIONS: Choose the one best response to each question.
III-1. For each patient choose the most likely peripheral blood smear:
(See Figure III-1)
FIGURE III-1 (see Color Atlas)
1. A 22-year-old man with a hematocrit of 17%. He has sickle cell disease and is admitted with a vaso-occlusive crisis after an upper respiratory illness.
2. A 36-year-old woman with a hematocrit of 32%. She had a splenectomy 5 years ago after a motor vehicle crash.
3. A 55-year-old man with a hematocrit of 28%. He has advanced alcoholic liver disease with cirrhosis and is awaiting liver transplantation.
4. A 64-year-old woman with a hematocrit of 28%. She has heme-positive stool and a 2-cm adenomatous colonic polyp at colonoscopy.
5. A 72-year-old man a hematocrit of 33%. Four years ago, he received a mechanical prosthetic aortic valve because of aortic stenosis caused by a congenital bicuspid valve.
III-2. A 39-year-old woman is evaluated for anemia. Her laboratory studies reveal a hemoglobin of 7.4 g/dL, hematocrit of 23.9%, mean corpuscular volume of 72 fL, mean cell hemoglobin of 25 pg, and mean cell hemoglobin concentration of 28%. The peripheral smear is shown in Figure III-2. Which of the following tests is most likely to be abnormal in this patient?
FIGURE III-2 (see Color Atlas)
C. Hemoglobin electrophoresis
D. Glucose-6-phosphate dehydrogenase
E. Vitamin B12
III-3. A 62-year-old man is evaluated for anemia. He has a hemoglobin of 9.0 g/dL (normal hemoglobin value, 15 g/dL), hematocrit of 27.0% (normal hematocrit, 45%), mean cell volume of 88 fL, mean cell hemoglobin of 28 pg, and mean cell hemoglobin concentration of 30%. On peripheral blood smear, polychromatophilic macrocytes are seen. The reticulocyte count is 9%. What is the reticulocyte production index?
III-4. You are asked to review the peripheral blood smear from a patient with anemia (Figure III-4). Serum lactate dehydrogenase is elevated, and there is hemoglobinuria. This patient is likely to have which physical examination finding?
FIGURE III-4 (see Color Atlas)
B. Heme-positive stools
C. Mechanical second heart sound
E. Thickened calvarium
III-5. In general, which of the following is the greatest risk factor for the development of cancer?
B. Alcohol use
C. Cigarette smoking
D. Female sex
III-6. Among women younger than 60 years of age who die from cancer, which of the following is the most common primary organ of origin?
D. Bone marrow
III-7. A 68-year-old woman is diagnosed with stage II breast cancer. She has a history of severe chronic obstructive pulmonary disease with an FEV1 of 32% predicted, coronary artery disease with prior stenting of the left anterior descending artery, peripheral vascular disease, and obesity. She continues to smoke 1 to 2 packs of cigarettes every day. She requires oxygen at 2 L/min continuously and is functionally quite limited. She currently is able to attend to all of her activities of daily living, including showering and dressing. She retired from her work as a waitress 10 years previously because of her lung disease. At home, she does attend to some of the household chores but is not able to use a vacuum. She goes out once or twice weekly to run typical errands and drives. She feels short of breath with most of these activities and often uses a motorized chart when out and about. How would you categorize her performance status and prognosis for treatment taking this into consideration?
A. She has an Eastern Cooperative Oncology Group (ECOG) grade of 1 and has a good prognosis with appropriate therapy.
B. She has an ECOG grade of 2 and has a good prognosis with appropriate therapy.
C. She has an ECOG grade of 3 and has a good prognosis with appropriate therapy.
D. She has an ECOG grade of 3 and has a poor prognosis despite therapy.
E. She has an ECOG grade of 4 and has a poor prognosis that precludes therapy.
III-8. Which of the following tumor markers is appropriately matched with the cell type cancer and can be followed during treatment as an adjunct to assess disease burden?
A. CA-125—Colon cancer
B. Calcitonin—Follicular carcinoma of the thyroid
C. CD30—Hairy cell leukemia
D. Human chorionic gonadotropin—Gestational trophoblastic disease
E. Neuron-specific enolase—Non–small cell carcinoma of the lung
III-9. Which of the following statements regarding current understanding of the genetic changes that must occur for a cell to become cancerous is TRUE?
A. Caretaker genes determine when a cell enters into a replicative phase and must acquire mutations to allow unregulated cell growth.
B. For a cell to become cancerous, it is estimated that a minimum of 20 mutations must occur.
C. For a tumor suppressor gene to become inactivated and allow unregulated cell growth, both copies of the gene must have mutations.
D. Oncogenes act in an autosomal recessive fashion.
E. Within a cancer, there are generally two to five cells of origin.
III-10. All the following conditions are associated with an increased incidence of cancer EXCEPT:
A. Down syndrome
B. Fanconi’s anemia
C. von Hippel–Lindau syndrome
E. Fragile X syndrome
III-11. Cancer therapy is increasingly personalized with targeted small molecule therapies that are directed against specific signal transduction pathways that are commonly activated in a particular cell type of cancer. Which of the following therapies is correctly matched with its molecular target?
III-12. Which of the following defines the term epigenetics?
A. Changes that alter the pattern of gene expression caused by mutations in the DNA code
B. Changes that alter the pattern of gene expression that persist across at least one cell division but are not caused by changes in the DNA code
C. Irreversible changes of the chromatin structure that regulates gene transcription and cell proliferation without permanent alteration of the DNA code
III-13. Which of the following patients with metastatic disease is potentially curable by surgical resection?
A. A 24-year-old man with a history of osteosarcoma of the left femur with a 1-cm metastasis to his right lower lobe referred for right lower lobectomy
B. A 56-year-old woman with a history of colon cancer with three metastases to the left lobe of the liver referred for left hepatic lobectomy
C. A 72-year-old man with metastatic prostate cancer to several vertebrae referred for orchiectomy
D. All of the above
E. None of the above
III-14. You are studying a new chemotherapeutic agent for use in advanced colorectal carcinoma and have completed a phase II clinical trial. Which of the following factors indicates that the drug is suitable for study in a phase III clinical trial?
A. Complete response rates of 10% to 15%
B. Increased disease-free survival rates by 1 month
C. Increased overall survival by 1 month
D. Partial response rate of 20% to 25%
E. Partial response rates of 50% or more
III-15. Match the following chemotherapeutic agents with their mechanisms of action:.
III-16. A 48-year-old woman with stage III breast cancer is undergoing chemotherapy with a regimen that includes doxorubicin. She presents 8 days after her last treatment to the emergency department with a fever of 104.1°F (40.1°C). She has chills, rigors, and a headache. Her chest radiograph, urinalysis, and tunneled intravenous catheter site show no obvious evidence of infection. Her white blood cell count upon presentation is 500/μL (0% neutrophils, 50% monocytes, 50% lymphocytes). Blood cultures are drawn peripherally and through the catheter. What is the next step in the treatment of this patient?
A. Broad-spectrum antibiotics with ceftazidime and vancomycin
B. Broad-spectrum antibiotics with ceftazidime, vancomycin, and voriconazole
C. Granulocyte-macrophage colony-stimulating factor after subsequent cycles of chemotherapy only
D. Granulocyte-macrophage colony-stimulating factor now and after subsequent cycles of chemotherapy
E. A and C
F. A and D
III-17. What is the most common side effect of chemotherapy?
C. Febrile neutropenia
E. Nausea with or without vomiting
III-18. A 24-year-old woman is seen in follow-up 12 months after an allogeneic stem cell transplant for acute myeloid leukemia. She is doing well without evidence of recurrent disease but has had manifestations of chronic graft-versus-host disease. She should be administered all of the following vaccines EXCEPT:
C. Measles, mumps, and rubella
D. Poliomyelitis via injection
E. 23-Valent pneumococcal polysaccharide
III-19. A 66-year-old woman has chronic lymphocytic leukemia with a stable white blood cell count of between 60,000 and 70,000/μL. She is currently hospitalized with pneumococcal pneumonia. This is the patient’s third episode of pneumonia within the past 12 months. What finding on laboratory testing would be most likely in this patient?
C. Impaired T-cell function with normal T-lymphocyte counts
D. Low CD4 count
E. No specific abnormality is expected.
III-20. A 63-year-old man is treated with chemotherapy for stage IIIB adenocarcinoma of the lung with paclitaxel and carboplatin. He presents for evaluation of a fever of 38.3°C (100.9°F). He is found to have erythema at the exit site of his tunneled catheter, although the tunnel itself is not tender or red. Blood cultures are negative at 48 hours. His neutrophil count is 1,550/μL. What is the best approach to the management of this patient?
A. Removal of catheter alone
B. Treatment with ceftazidime and vancomycin
C. Treatment with topical antibiotics at the catheter site
D. Treatment with vancomycin alone
E. Treatment with vancomycin and removal of catheter
III-21. A 48-year-old woman presents to her physician with a complaint of an enlarging mole on her right lower extremity. She had noticed the area about 1 year previously and believes it has enlarged. She also notes that it recently has become itchy and occasionally bleeds. On physical examination, the lesion is located on the right mid-thigh. It measures 7.5 × 6 mm with irregular borders and a variegated hue with some areas appearing quite black. A biopsy confirms nodular melanoma. Which of the following is the best predictor of metastatic risk in this patient?
A. Breslow thickness
B. Clark level
C. Female gender
D. Presence of ulceration
E. Site of lesion
III-22. A 53-year-old man with a history of superficial spreading melanoma is diagnosed with disease metastatic to the lungs and bones. Genetic testing confirms the presence of the BRAF V600E mutation. What do you recommend for treatment of this patient?
B. Hospice care
III-23. A 65-year-old man presents to his primary care physician complaining of a hoarse voice for 6 months. He smokes 1 pack of cigarettes daily and drinks at least a six pack of beer daily. His physical examination reveals a thin man with a weak voice in no distress. No stridor is heard. The head and neck examination is normal. No cervical lymphadenopathy is present. He is referred to otolaryngology where a laryngeal lesion is discovered. Biopsy reveals squamous cell carcinoma. On imaging, the mass measures 2.8 cm. No suspicious lymphadenopathy is present on PET imaging. What is the best choice of therapy in this patient?
A. Concomitant chemotherapy and radiation therapy
B. Chemotherapy alone
C. Radiation therapy alone
D. Radical neck dissection alone
E. Radical neck dissection followed by concomitant chemotherapy and radiation
III-24. Which of the following statements is true with regard to the solitary pulmonary nodule?
A. A lobulated and irregular contour is more indicative of malignancy than a smooth one.
B. About 80% of incidentally found pulmonary nodules are benign.
C. Absence of growth over a period of 6 to 12 months is sufficient to determine if a solitary pulmonary nodule is benign.
D. Ground-glass nodules should be regarded as benign.
E. Multiple nodules indicate malignant disease.
III-25. A 64-year-old man seeks evaluation for a solitary pulmonary nodule that was found incidentally. He had presented to the emergency department for shortness of breath and chest tightness. A CT pulmonary angiogram did not show any evidence of pulmonary embolism. However, a 9-mm nodule is seen in the periphery of the left lower lobe. No enlarged mediastinal lymph nodes are present. He is a current smoker of 2 packs of cigarettes daily and has done so since the 16 years of age. He generally reports no functional limitation related to respiratory symptoms. His FEV1 is 88% predicted, FVC is 92% predicted, and diffusion capacity is 80% predicted. He previously had a normal chest x-ray 3 years previously. What is the next best step in the evaluation and treatment of this patient?
A. Perform a bronchoscopy with biopsy for diagnosis.
B. Perform a combined PET and CT to assess for uptake in the nodule and assess for lymph node metastases.
C. Perform a follow-up CT scan in 3 months to assess for interval growth.
D. Refer the patient to radiation oncology for stereotactic radiation of the dominant nodule.
E. Refer the patient to thoracic surgery for video-assisted thoracoscopic biopsy and resection of lung nodule if malignancy is diagnosed.
III-26. A 62-year-old man presents to the emergency department complaining of a droopy right eye and blurred vision for the past day. The symptoms started abruptly, and he denies any antecedent illness. For the past 4 months, he has been complaining of increasing pain in his right arm and shoulder. His primary care physician has treated him for shoulder bursitis without relief. His medical history is significant for COPD and hypertension. He smokes 1 pack of cigarettes daily. He has chronic daily sputum production and has stable dyspnea on exertion. On physical examination, he has right eye ptosis with unequal pupils. On the right, his pupil is 2 mm and not reactive; on the left, the pupil is 4 mm and reactive. However, his ocular movements appear intact. His lung fields are clear to auscultation. On extremity examination, there is wasting of the intrinsic muscles of the hand. Which of the following would be most likely to explain the patient’s constellation of symptoms?
A. Enlarged mediastinal lymph nodes causing occlusion of the superior vena cava
B. Metastases to the midbrain from small cell lung cancer
C. Paraneoplastic syndrome caused by antibodies to voltage-gated calcium channels
D. Presence of a cervical rib on chest radiography
E. Right apical pleural thickening with a mass-like density measuring 1 cm in thickness
III-27. A 55-year-old man presents with superior vena cava syndrome and is diagnosed with small cell lung cancer. Which of the following tests are indicated to properly stage this patient?
A. Bone marrow biopsy
B. CT scan of the abdomen
C. CT or MRI of the brain with intravenous contrast
D. Lumbar puncture
E. B and C
F. All of the above
III-28. As an oncologist you are considering treatment options for your patients with lung cancer, including small molecule therapy targeting the epidermal growth factor receptor (EGFR). Which of the following patients is most likely to have an EGFR mutation?
A. A 23-year-old man with a hamartoma
B. A 33-year-old woman with a carcinoid tumor
C. A 45-year-old woman who has never smoked with an adenocarcinoma
D. A 56-year-old man with a 100 pack-year history of tobacco with small cell lung carcinoma
E. A 76-year-old man with squamous cell carcinoma and a history of asbestos exposure
III-29. Given that most individuals with lung cancer present with advanced disease and have a high mortality, much research has investigated methods for early detection of lung cancer. Which of the following approaches is most likely to impact disease-related mortality from lung cancer?
A. Carefully design and implement low-dose chest CT screening in individuals with greater than 30 pack years of cigarette smoking
B. Continue annual screening with a chest x-ray for individuals with greater than 30 pack years of cigarette smoking
C. Do not recommend any screening because 30 years of research has not demonstrated any effect on mortality from lung cancer
D. Offer screening with low-dose CTs to all current or former smokers
E. Offer screening with combined PET and CT to individuals with greater than 30 pack years of tobacco use
III-30. A 34-year-old woman is seen by her internist for evaluation of right breast mass. This was noted approximately 1 week ago when she was showering. She has not had any nipple discharge or discomfort. She has no other medical problems. On examination, her right breast has a soft 1 cm × 2 cm mass in the right upper quadrant. There is no axillary lymphadenopathy present. The contralateral breast is normal. The breast is reexamined in 3 weeks, and the same findings are present. The cyst is aspirated, and clear fluid is removed. The mass is no longer palpable. Which of the following statements is true?
A. Breast MRI should be obtained to discern for residual fluid collection.
B. Mammography is required to further evaluate the lesion.
C. She should be evaluated in 1 month for recurrence.
D. She should be referred to a breast surgeon for resection.
E. She should not breastfeed any more children.
III-31. Which of the following women has the lowest risk of breast cancer?
A. A woman with menarche at 12 years, first child at 24 years, and menopause at 47 years
B. A woman with menarche at 14 years, first child at 17 years, and menopause at 52 years
C. A woman with menarche at 16 years, first child at 17 years, and menopause at 42 years
D. A woman with menarche at 16 years, first child at 32 years, and menopause at 52 years
E. They are all equal
III-32. Which of the following tumor characteristics confers a poor prognosis in patients with breast cancer?
A. Estrogen receptor positive
B. Good nuclear grade
C. Low proportion of cells in S-phase
D. Overexpression of erbB2 (HER-2/neu)
E. Progesterone receptor positive
III-33. A 56-year-old man presents to a physician with weight loss and dysphagia. He feels that food gets stuck in his mid-chest such that he no longer is able to eat meats. He reports his diet consists primarily of soft foods and liquids. The symptoms have progressively worsened over 6 months. During this time, he has lost about 50 lb. He occasionally gets pain in his mid-chest that radiates to his back and also occasionally feels that he regurgitates undigested foods. He does not have a history of gastroesophageal reflux disease. He does not regularly seek medical care. He is known to have hypertension but takes no medications. He drinks 500 cc or more of whiskey daily and smokes 1.5 packs of cigarettes per day. On physical examination, the patient appears cachectic with temporal wasting. He has a body mass index of 19.4 kg/m2. His blood pressure is 198/110 mm Hg, heart rate is 110 beats/min, respiratory rate is 18 breaths/min, temperature is 37.4°C (99.2°F), and oxygen saturation is 93% on room air. His pulmonary examination shows decreased breath sounds at the apices with scattered expiratory wheezes. His cardiovascular examination demonstrates an S4 gallop with a hyperdynamic precordium. A regular tachycardia is present. Blood pressures are equal in both arms. Liver span is not enlarged. There are no palpable abdominal masses. What is the most likely cause of the patient’s presentation?
A. Adenocarcinoma of the esophagus
B. Ascending aortic aneurysm
C. Esophageal stricture
D. Gastric cancer
E. Squamous cell carcinoma of the esophagus
III-34. A 64-year-old woman presents with complaints of a change in stool caliber for the past 2 months. The stools now have a diameter of only the size of her fifth digit. Over this same period, she feels she has to exert increasing strain to have a bowel movement and sometimes has associated abdominal cramping. She often has blood on the toilet paper when she wipes. During this time, she has lost about 20 lb. On physical examination, the patient appears cachectic with a body mass index of 22.5 kg/m2. The abdomen is flat and nontender. The liver span is 12 cm to percussion. On digital rectal examination, a mass lesion is palpated approximately 8 cm into the rectum. A colonoscopy is attempted, which demonstrates a 2.5-cm sessile mass that narrows the colonic lumen. The biopsy confirms adenocarcinoma. The colonoscope is not able to traverse the mass. A CT scan of the abdomen does not show evidence of metastatic disease. Liver function test results are normal. A carcinoembryonic antigen level is 4.2 ng/mL. The patient is referred for surgery and undergoes rectosigmoidectomy with pelvic lymph node dissection. Final pathology demonstrates extension of the primary tumor into the muscularis propria but not the serosa. Of 15 lymph nodes removed, two are positive for tumor. What do you recommend for this patient after surgery?
A. Chemotherapy with a regimen containing 5-fluorouracil
B. Complete colonoscopy within 3 months
C. Measurement of CEA levels at 3-month intervals
D. Radiation therapy to the pelvis
E. All of the above
III-35. A healthy 62-year-old woman returns to your clinic after undergoing routine colonoscopy. Findings included two 1.3-cm sessile (flat-based), villous adenomas in her ascending colon that were removed during the procedure. What is the next step in management?
A. Colonoscopy in 3 years
B. Colonoscopy in 10 years
C. CT scan of the abdomen
D. Partial colectomy
III-36. Which of the following should prompt investigation for hereditary nonpolyposis colon cancer screening in a 32-year-old man?
A. Father, paternal aunt, and paternal cousin with colon cancer with ages of diagnosis of 54, 68, and 37 years, respectively
B. Innumerable polyps visualized on routine colonoscopy
C. Mucocutaneous pigmentation
D. New diagnosis of ulcerative colitis
E. None of the above
III-37. All of the following statements regarding pancreatic cancer are true EXCEPT:
A. Alcohol consumption is not a risk factor for pancreatic cancer.
B. Cigarette smoking is a risk factor for pancreatic cancer.
C. Despite accounting for fewer than 5% of malignancies diagnosed in the United States, pancreatic cancer is the fourth leading cause of cancer death.
D. If detected early, the 5-year survival is up to 20%.
E. The 5-year survival rates for pancreatic cancer have improved substantially in the past decade.
III-38. A 65-year-old man is evaluated in clinic for 1 month of progressive painless jaundice and 10 lb of unintentional weight loss. His physical examination is unremarkable. A dual-phase contrast CT shows a suspicious mass in the head of the pancreas with biliary ductal dilation. Which of the following is the best diagnostic test to evaluate for suspected pancreatic cancer?
A. CT-guided percutaneous needle biopsy
B. Endoscopic ultrasound-guided needle biopsy
C. ERCP with pancreatic juice sampling for cytopathology
D. FDG-PET imaging
E. Serum CA 19-9
III-39. A 63-year-old man complains of notable pink-tinged urine for the past month. At first he thought it was caused by eating beets, but has not cleared. His medical history is notable for hypertension and cigarette smoking. He does report some worsening urinary frequency and hesitancy over the past 2 years. Physical examination is unremarkable. Urinalysis is notable for gross hematuria with no white blood cells or casts. Renal function is normal. Which of the following statements regarding this patient is true?
A. Cigarette smoking is not a risk for bladder cancer.
B. Gross hematuria makes prostate cancer more likely than bladder cancer.
C. If invasive bladder cancer with nodal involvement but no distant metastases is found, the 5-year survival is 20%.
D. If superficial bladder cancer is found, intravesicular BCG may be used as adjuvant therapy.
E. Radical cystectomy is generally recommended for invasive bladder cancer.
III-40. A 68-year-old man comes to his physician complaining of 2 months of increasing right flank pain with 1 month of worsening hematuria. He was treated for cystitis at a walk-in clinic 3 weeks ago with no improvement. He also reports poor appetite and 5 lb of weight loss. His physical examination is notable for a palpable mass in the right flank measuring greater than 5 cm. His renal function is normal. All of the following are true about this patient’s likely diagnosis EXCEPT:
A. Anemia is more common than erythrocytosis.
B. Cigarette smoking increased his risk.
C. If his disease has metastasized, with best therapy 5-year survival is greater than 50%.
D. If his disease is confined to the kidney, 5-year survival is greater than 80%.
E. The most likely pathology is clear cell carcinoma.
III-41. In the patient described above, imaging shows a 10-cm solid mass in the right kidney and multiple nodules in the lungs consistent with metastatic disease. Needle biopsy of a lung lesion confirms the diagnosis of renal cell carcinoma. Which of the following is recommended therapy?
D. Radical nephrectomy
III-42. Which of the following has been shown in randomized trials to reduce the future risk of pancreatic cancer diagnosis?
D. Vitamin C
E. Vitamin E
III-43. A 54-year-old man is evaluated in an executive health program. On physical examination, he is noted to have an enlarged prostate with a right lobe nodule. He does not recall his last digital rectal examination and has never had prostate-specific antigen (PSA) tested. Based on this evaluation, which of the following is next recommended?
A. Bone scan to evaluate for metastasis
C. PSA now and in 3 months to measure PSA velocity
D. Repeat digital rectal examination in 3 months
E. Transrectal ultrasound-guided biopsy
III-44. Which of the following statements describes the relationship between testicular tumors and serum markers?
A. Pure seminomas produce α-fetoprotein (AFP) or beta human chorionic gonadotropin (β-hCG) in more than 90% of cases.
B. More than 40% of nonseminomatous germ cell tumors produce no cell markers.
C. Both β-hCG and AFP should be measured in following the progress of a tumor.
D. Measurement of tumor markers the day after surgery for localized disease is useful in determining the completeness of the resection.
E. β-hCG is limited in its usefulness as a marker because it is identical to human luteinizing hormone.
III-45. A 32-year-old man presents complaining of a testicular mass. On examination, you palpate a 1 cm × 2 cm painless mass on the surface of the left testicle. A chest x-ray shows no lesions, and a CT scan of the abdomen and pelvis shows no evidence of retroperitoneal adenopathy. The α-fetoprotein (AFP) level is elevated at 400 ng/mL. Beta human chorionic gonadotropin (β-hCG) is normal, as is lactate dehydrogenase (LDH). You send the patient for an orchiectomy. The pathology comes back as seminoma limited to the testis alone. The AFP level declines to normal at an appropriate interval. What is the appropriate management at this point?
A. Radiation to the retroperitoneal lymph nodes
B. Adjuvant chemotherapy
C. Hormonal therapy
D. Retroperitoneal lymph node dissection (RPLND)
E. Positron emission tomography (PET) scan
III-46. Which of the following statements regarding the relationship between ovarian cancer and BRCA gene mutations is true?
A. Most women with BRCA mutations have a family history that is strongly positive for breast or ovarian cancer (or both).
B. More than 30% of women with ovarian cancer have a somatic mutation in either BRCA1 or BRCA2.
C. Prophylactic oophorectomy in patients with BRCA mutations does not protect against the development of breast cancer.
D. Screening studies with serial ultrasound and serum CA-125 tumor marker studies are effective in detecting early stage disease.
E. Women with known mutations in a single BRCA1 or BRCA2 allele have a 75% lifetime risk of developing ovarian cancer.
III-47. All of the following statements regarding the diagnosis of uterine cancer are true EXCEPT:
A. Five-year survival after surgery in disease confined to the corpus is approximately 90%.
B. Endometrial carcinoma is the most common gynecologic malignancy in the United States.
C. Most women present with amenorrhea.
D. Tamoxifen is associated with an increased risk of endometrial carcinoma.
E. Unopposed estrogen exposure is a risk factor for developing endometrial carcinoma.
III-48. A 73-year-old man presents to the clinic with 3 months of increasing back pain. He localizes the pain to the lumbar spine and states that the pain is worst at night while he is lying in bed. It is improved during the day with mobilization. Past history is notable only for hypertension and remote cigarette smoking. Physical examination is normal. Laboratory studies are notable for an elevated alkaline phosphatase. A lumbar radiogram shows a lytic lesion in the L3 vertebra. Which of the following malignancies is most likely?
A. Gastric carcinoma
B. Non–small cell lung cancer
D. Pancreatic carcinoma
E. Thyroid carcinoma
III-49. A primary tumor of which of these organs is the least likely to metastasize to bone?
III-50. A 22-year-old man comes into clinic because of a swollen leg. He does not remember any trauma to the leg, but the pain and swelling began 3 weeks ago in the anterior shin area of his left foot. He is a college student and is active in sports daily. A radiograph of the right leg shows a destructive lesion with a “moth-eaten” appearance extending into the soft tissue and a spiculated periosteal reaction. Codman’s triangle (a cuff of periosteal bone formation at the margin of the bone and soft tissue mass) is present. Which of the following are the most likely diagnosis and optimal therapy for this lesion?
A. Chondrosarcoma; chemotherapy alone is curative
B. Chondrosarcoma; radiation with limited surgical resection
C. Osteosarcoma; preoperative chemotherapy followed by limb-sparing surgery
D. Osteosarcoma; radiation therapy
E. Plasma cell tumor; chemotherapy
III-51. A 42-year-old man presented to the hospital with right upper quadrant pain. He was found to have multiple masses in the liver that were found to be malignant on H&E staining of a biopsy sample. Your initial history, physical examination, and laboratory tests, including prostate-specific antigen, are unrevealing. Lung, abdominal, and pelvic CT scans are unremarkable. He is an otherwise healthy individual with no chronic medical problems. Which immunohistochemical markers should be obtained from the biopsy tissue?
C. Leukocyte common antigen
E. Thyroid transcription factor 1
III-52. A 52-year-old woman is evaluated for abdominal swelling with a computed tomogram that shows ascites and likely peritoneal studding of tumor but no other abnormality. Paracentesis shows adenocarcinoma but cannot be further differentiated by the pathologist. A thorough physical examination, including breast and pelvic examination, shows no abnormality. CA-125 levels are elevated. Pelvic ultrasonography and mammography findings are normal. Which of the following statements is true?
A. Compared with other women with known ovarian cancer at a similar stage, this patient can be expected to have a less than average survival.
B. Debulking surgery is indicated.
C. Surgical debulking plus cisplatin and paclitaxel is indicated.
D. Bilateral mastectomy and bilateral oophorectomy will improve survival.
E. Fewer than 1% of patients with this disorder will remain disease free 2 years after treatment.
III-53. A 29-year-old man is found on routine chest radiography for life insurance to have left hilar adenopathy. CT scanning confirms enlarged left hilar and paraaortic nodes. He is otherwise healthy. Besides biopsy of the lymph nodes, which of the following is indicated?
A. Angiotensin-converting enzyme (ACE) level
C. Thyroid-stimulating hormone (TSH)
E. C-reactive protein
III-54. An 81-year-old man is admitted to the hospital for altered mental status. He was found at home, confused and lethargic, by his son. His medical history is significant for metastatic prostate cancer. The patient’s medications include periodic intramuscular goserelin injections. On examination, he is afebrile. Blood pressure is 110/50 mm Hg, and the pulse rate is 110 beats/min. He is lethargic and minimally responsive to sternal rub. He has bitemporal wasting, and his mucous membranes are dry. On neurologic examination, he is obtunded. The patient has an intact gag reflex and withdraws to pain in all four extremities. Rectal tone is normal. Laboratory values are significant for a creatinine of 4.2 mg/dL, a calcium level of 14.4 meq/L, and an albumin of 2.6 g/dL. All the following are appropriate initial management steps EXCEPT:
A. Normal saline
C. Furosemide when the patient is euvolemic
III-55. A 55-year-old man is found to have a serum calcium of 13.0 mg/dL after coming to clinic complaining of fatigue and thirst for the past month. A chest radiograph demonstrates a 4-cm mass in the right lower lobe. Which of the following serum tests is most likely to reveal the cause of his hypercalcemia?
A. Adrenocorticotropic hormone (ACTH)
B. Antidiuretic hormone (ADH)
C. Insulin-like growth factor
D. Parathyroid hormone (PTH)
E. Parathyroid hormone related protein (PTH-rp)
III-56. A 55-year-old woman presents with progressive incoordination. Physical examination is remarkable for nystagmus, mild dysarthria, and past pointing on finger-to-nose testing. She also has an unsteady gait. MRI reveals atrophy of both lobes of the cerebellum. Sero-logic evaluation reveals the presence of anti-Yo antibody. Which of the following is the most likely cause of this clinical syndrome?
A. Non–small cell cancer of the lung
B. Small cell cancer of the lung
C. Breast cancer
D. Non-Hodgkin’s lymphoma
E. Colon cancer
III-57. All of the following conditions may be associated with a thymoma EXCEPT:
C. Myasthenia gravis
E. Pure red blood cell aplasia
III-58. A 52-year-old woman has been having worsening cough for the past month. She is a nonsmoker and has no known health problems. The cough is nonproductive and present throughout the day and night. It worsens when lying on her back. She has also noticed some upper chest pain and dyspnea on exertion for the past week. A chest radiograph shows a large mass (>5 cm) confined to the anterior mediastinum. Which of the following diagnoses is most likely?
A. Hodgkin’s lymphoma
B. Non-Hodgkin’s lymphoma
E. Thyroid carcinoma
III-59. All the following are suggestive of iron-deficiency anemia EXCEPT:
C. Decreased serum ferritin
D. Decreased total iron-binding capacity (TIBC)
E. Low reticulocyte response
III-60. A 24-year-old man with a history of poorly treated chronic ulcerative colitis is found to have anemia with a hemoglobin of 9 g/dL and a reduced mean corpuscular volume. His ferritin is 250 μg/L. Which of the following is the most likely cause of his anemia?
A. Folate deficiency
D. Iron deficiency
E. Sideroblastic anemia
III-61. All of the following statements regarding the anemia of chronic kidney disease are true EXCEPT:
A. The degree of anemia correlates with the stage of chronic kidney disease.
B. Erythropoietin levels are reduced.
C. Ferritin is reduced.
D. It is typically normocytic and normochromic.
E. Reticulocytes are decreased.
III-62. All of the following statements regarding the utility of hydroxyurea in patients with sickle cell disease are true EXCEPT:
A. It is effective in reducing painful crises.
B. It produces a chimeric state with partial production of hemoglobin A by the bone marrow.
C. It should be considered in patients with repeated acute chest syndrome episodes.
D. Its mechanism involves increasing production of fetal hemoglobin.
E. The major adverse effect is a reduction in white blood cell count.
III-63. Which of the following is the most cost-effective test to evaluate a patient for suspected cobalamin (vitamin B12) deficiency?
A. Red blood cell folate
B. Serum cobalamin
C. Serum homocysteine
D. Serum methylmalonate
E. Serum pepsinogen
III-64. A patient being evaluated for anemia has the peripheral blood smear shown in Figure III-64. Which of the following is the most likely cause of the anemia?
FIGURE III-64 (see Color Atlas)
A. Acute lymphocytic leukemia
B. Autoantibodies to ADAMTS-13
C. Cobalamin deficiency
D. Epstein-Barr virus infection
E. Iron deficiency
III-65. Patients from which of the following regions need not be screened for glucose-6-phosphate dehydrogenase (G6PD) deficiency when starting a drug that carries a risk for G6PD-mediated hemolysis?
C. Southeast Asia
D. Southern Europe
E. Sub-Saharan Africa
F. None of the above
III-66. A 36-year-old African American woman with systemic lupus erythematosus presents with the acute onset of lethargy and jaundice. On initial evaluation, she is tachycardic and hypotensive, appears pale, is dyspneic, and is somewhat difficult to arouse. Physical examination reveals splenomegaly. Her initial hemoglobin is 6 g/dL, white blood cell count is 6300/μL, and platelets are 294,000/μL. Her total bilirubin is 4 g/dL, reticulocyte count is 18%, and haptoglobin is not detectable. Renal function is normal, as is urinalysis. What would you expect on her peripheral blood smear?
A. Macrocytosis and polymorphonuclear leukocytes with hypersegmented nuclei
D. Sickle cells
E. Target cells
III-67. A 22-year-old pregnant woman of northern European descent presents 3 months into her first pregnancy with extreme fatigue, pallor, and icterus. She reports being previously healthy. On evaluation her hemoglobin is 8 g/dL with a normal mean corpuscular volume and an elevated mean corpuscular hemoglobin concentration, reticulocyte count is 9%, indirect bilirubin is 4.9 mg/dL, and serum haptoglobin is not detectable. Her peripheral smear is shown in Figure III-67. Her physical examination is notable for splenomegaly and a normal 3-month uterus. What is the most likely diagnosis?
FIGURE III-67 (see Color Atlas)
A. Colonic polyp
B. G6PD deficiency
C. Hereditary spherocytosis
D. Parvovirus B19 infection
E. Thrombotic thrombocytopenic purpura
III-68. The triad of portal vein thrombosis, hemolysis, and pancytopenia suggests which of the following diagnoses?
A. Acute promyelocytic leukemia
B. Hemolytic uremic syndrome (HUS)
D. Paroxysmal nocturnal hemoglobinuria (PNH)
E. Thrombotic thrombocytopenic purpura (TTP)
III-69. All of the following laboratory values are consistent with an intravascular hemolytic anemia EXCEPT:
A. Increased haptoglobin
B. Increased lactate dehydrogenase (LDH)
C. Increased reticulocyte count
D. Increased unconjugated bilirubin
E. Increased urine hemosiderin
III-70. Which of the following hemolytic anemias can be classified as extracorpuscular?
B. Paroxysmal nocturnal hemoglobinuria
C. Pyruvate kinase deficiency
D. Sickle cell anemia
E. Thrombotic thrombocytopenic purpura
III-71. A 34-year-old woman with a medical history of sickle cell anemia presents with a 5-day history of fatigue, lethargy, and shortness of breath. She denies chest pain and bone pain. She has had no recent travel. Of note, the patient’s 4-year-old daughter had a “cold” 2 weeks before the presentation. On examination, the woman has pale conjunctiva, is anicteric, and is mildly tachycardic. Abdominal examination is unremarkable. Laboratory studies show a hemoglobin of 3 g/dL; her baseline is 8 g/dL. The white blood cell count and platelets are normal. Reticulocyte count is undetectable. Total bilirubin is 1.4 mg/dL. Lactic dehydrogenase is at the upper limits of the normal range. Peripheral blood smear shows a few sickled cells but a total absence of reticulocytes. The patient is given a transfusion of 2 units of packed red blood cells and admitted to the hospital. A bone marrow biopsy shows a normal myeloid series but an absence of erythroid precursors. Cytogenetics are normal. What is the most appropriate next management step?
A. Make arrangements for exchange transfusion.
B. Tissue type her siblings for a possible bone marrow transplant.
C. Check parvovirus titers.
D. Start prednisone and cyclosporine.
E. Start broad-spectrum antibiotics.
III-72. Aplastic anemia has been associated with all of the following EXCEPT:
A. Carbamazepine therapy
B. Methimazole therapy
C. Nonsteroidal anti-inflammatory drugs
D. Parvovirus B19 infection
E. Seronegative hepatitis
III-73. A 23-year-old man presents with diffuse bruising. He otherwise feels well. He takes no medications, does not use dietary supplements, and does not use illicit drugs. His medical history is negative for any prior illnesses. He is a college student and works as a barista in a coffee shop. A blood count reveals an absolute neutrophil count of 780/μL, hematocrit of 18%, and platelet count of 21,000/μL. Bone marrow biopsy reveals hypocellularity with a fatty marrow. Chromosome studies of peripheral blood and bone marrow cells are performed that exclude Fanconi’s anemia and myelodysplastic syndrome. The patient has a fully histocompatible brother. Which of the following is the best therapy?
A. Antithymocyte globulin plus cyclosporine
C. Growth factors
D. Hematopoietic stem cell transplant
E. Red blood cell and platelet transfusion
III-74. A 73-year-old man has complained of fatigue and worsening dyspnea on exertion for the past 2 to 3 months. His medical history is only notable for hypertension and hypercholesterolemia. He is an active golfer who notes that lately he has difficulty walking 18 holes. His handicap has increased by 5 strokes over this time period. Physical examination reveals normal vital signs and is unremarkable except for pallor. His laboratory examination is remarkable for a hematocrit of 25% with a platelet count of 185,000/μL and low normal white cell count. There are no circulating blasts. These abnormalities were not present 1 year ago. Bone marrow reveals a hypercellular marrow with fewer than 5% blasts and the 5q-cytogenetic abnormality. All of the following statements regarding this patient’s condition are true EXCEPT:
A. He has myelofibrosis.
B. He is most likely to die as a result of leukemic transformation.
C. His median survival is more than 12 months.
D. Lenalidomide is effective in reversing the anemia.
E. Only stem cell transplantation offers a cure.
III-75. All of the following are considered myeloproliferative disorders in the WHO classification system EXCEPT:
A. Chronic myelogenous leukemia (bcr-abl positive)
B. Essential thrombocytosis
D. Polycythemia vera
E. Primary effusion lymphoma
III-76. Which of the following statements regarding polycythemia vera is correct?
A. An elevated plasma erythropoietin level excludes the diagnosis.
B. Transformation to acute leukemia is common.
C. Thrombocytosis correlates strongly with thrombotic risk.
D. Aspirin should be prescribed to all of these patients to reduce thrombotic risk.
E. Phlebotomy is used only after hydroxyurea and inter-feron have been tried.
III-77. A 68-year-old man seeks evaluation for fatigue, weight loss, and early satiety that have been present for about 4 months. On physical examination, his spleen is noted to be markedly enlarged. It is firm to touch and crosses the midline. The lower edge of the spleen reaches to the pelvis. His hemoglobin is 11.1 g/dL and hematocrit is 33.7%. The leukocyte count is 6200/μL and platelet count is 220,000/μL. The white cell count differential is 75% polymorphonuclear leukocytes, 8% myelocytes, 4% metamyelocytes, 8% lymphocytes, 3% monocytes, and 2% eosinophils. The peripheral blood smear shows teardrop cells, nucleated red blood cells, and immature granulocytes. Rheumatoid factor is positive. A bone marrow biopsy is attempted, but no cells are able to be aspirated. No evidence of leukemia or lymphoma is found. What is the most likely cause of the splenomegaly?
A. Chronic primary myelofibrosis
B. Chronic myelogenous leukemia
C. Rheumatoid arthritis
D. Systemic lupus erythematosus
III-78. A 50-year-old woman presents to your clinic for evaluation of an elevated platelet count. The latest complete blood count is white blood cells (WBC), 7000/μL; hematocrit, 34%; and platelets, 600,000/μL. All the following are common causes of thrombocytosis EXCEPT:
A. Iron-deficiency anemia
B. Essential thrombocytosis
C. Chronic myeloid leukemia
E. Pernicious anemia
III-79. A 38-year-old woman is referred for evaluation of an elevated hemoglobin and hematocrit that was discovered during an evaluation of recurrent headaches. Until about 8 months previously, she was in good health, but she developed increasingly persistent headaches with intermittent vertigo and tinnitus. She was originally prescribed sumatriptan for presumed migraine headaches but did not experience relief of her symptoms. A CT scan of the brain showed no evidence of mass lesion. During evaluation of her headaches, she was found to have a hemoglobin of 17.3 g/dL and a hematocrit of 52%. Her only other symptom is diffuse itching after hot showers. She is a nonsmoker. She has no history pulmonary or cardiac disease. On physical examination, she appears well. Her body mass index is 22.3 kg/m2. Vitals signs are blood pressure of 148/84 mm Hg, heart rate of 86 beats/min, respiratory rate of 12 breaths/min, and SaO2 of 99% on room air. She is afebrile. The physical examination, including full neurologic examination, is normal. There are no heart murmurs. There is no splenomegaly. Peripheral pulses are normal. Laboratory studies confirm elevated hemoglobin and hematocrit. She also has a platelet count of 650,000/μL. Leukocyte count is 12,600/μL with a normal differential. Which of the following tests should be performed next in the evaluation of this patient?
A. Bone marrow biopsy
B. Erythropoietin level
C. Genetic testing for JAK2 V617F mutation
D. Leukocyte alkaline phosphatase
E. Red blood cell mass and plasma volume determination
III-80. A 45-year-old man is evaluated by his primary care physician for complaints of early satiety and weight loss. On physical examination, his spleen is palpable 10 cm below the left costal margin and is mildly tender to palpation. His laboratory studies show a leukocyte count of 125,000/μL with a differential of 80% neutrophils, 9% bands, 3% myelocytes, 3% metamyelocytes, 1% blasts, 1% lymphocytes, 1% eosinophils, and 1% basophils. Hemoglobin is 8.4 g/dL, hematocrit is 26.8%, and platelet count is 668,000/μL. A bone marrow biopsy demonstrates increased cellularity with an increased myeloid to erythroid ratio. Which of the following cytogenetic abnormalities is most likely to be found in this patient?
A. Deletion of a portion of the long arm of chromosome 5, del(5q)
B. Inversion of chromosome 16, inv(16)
C. Reciprocal translocation between chromosomes 9 and 22 (Philadelphia chromosome)
D. Translocations of the long arms of chromosomes 15 and 17
E. Trisomy 12
III-81. All of the following statements regarding the epidemiology of and risk factors for acute myeloid leukemias are true EXCEPT:
A. Anticancer drugs such as alkylating agents and topoisomerase II inhibitors are the leading cause of drug-associated myeloid leukemias.
B. Individuals exposed to high-dose radiation are at risk for acute myeloid leukemia, but individuals treated with therapeutic radiation are not unless they are also treated with alkylating agents.
C. Men have a higher incidence of acute myeloid leukemia than women.
D. The incidence of acute myeloid leukemia is greatest in individuals younger than 20 years of age.
E. Trisomy 21 (Down syndrome) is associated with an increased risk of acute myeloid leukemia.
III-82. A 56-year-old woman is diagnosed with chronic myelogenous leukemia, Philadelphia chromosome positive. Her presenting leukocyte count was 127,000/μL, and her differential shows less than 2% circulating blasts. Her hematocrit is 21.1% at diagnosis. She is asymptomatic except for fatigue. She has no siblings. What is the best initial therapy for this patient?
A. Allogeneic bone marrow transplant
B. Autologous stem cell transplant
C. Imatinib mesylate
III-83. A 48-year-old woman is admitted to the hospital with anemia and thrombocytopenia after complaining of profound fatigue. Her initial hemoglobin is 8.5 g/dL, hematocrit is 25.7%, and platelet count is 42,000/μL. Her leukocyte count is 9540/μL, but 8% blast forms are noted on peripheral smear. A chromosomal analysis shows a reciprocal translocation of the long arms of chromosomes 15 and 17, t(15;17), and a diagnosis of acute promyelocytic leukemia is made. The induction regimen of this patient should include which of the following drugs?
B. Cyclophosphamide, daunorubicin, vinblastine, and prednisone
E. Whole-body irradiation
III-84. The patient in question III-83 is started on the appropriate induction regimen. Two weeks after initiation of treatment, the patient develops acute onset of shortness of breath, fever, and chest pain. Her chest radiograph shows bilateral alveolar infiltrates and moderate bilateral pleural effusions. Her leukocyte count is now 22,300/μL, and she has a neutrophil count of 78%, bands of 15%, and lymphocytes 7%. She undergoes bronchoscopy with lavage that shows no bacterial, fungal, or viral organisms. What is the most likely diagnosis in this patient?
A. Arsenic poisoning
B. Bacterial pneumonia
C. Cytomegalovirus pneumonia
D. Radiation pneumonitis
E. Retinoic acid syndrome
III-85. A 76-year-old man is admitted to the hospital with complaints of fatigue for 4 months and fever for the past 1 week. His temperature has been as high as 38.3°C at home. During this time, he intermittently has had a 5.5-kg weight loss, severe bruising with minimal trauma, and an aching sensation in his bones. He last saw his primary care physician 2 months ago and was diagnosed with anemia of unclear etiology at that time. He has a history of a previous left middle cerebral artery cerebrovascular accident, which has left him with decreased functional status. At baseline, he is able to ambulate in his home with the use of a walker and is dependent on a caregiver for assistance with his activities of daily living. His vital signs are blood pressure of 158/86 mm Hg, heart rate of 98 beats/min, respiratory rate of 18 breaths/min, SaO2 95%, and temperature of 38°C. He appears cachectic with temporal muscle wasting. He has petechiae on his hard palate. He has no lymph node enlargement. On cardiovascular examination, there is a II/VI systolic ejection murmur present. His lungs are clear. The liver is enlarged and palpable 6 cm below the right costal margin. In addition, the spleen is also enlarged, with a palpable spleen tip felt about 4 cm below the left costal margin. There are multiple hematomas and petechiae present in the extremities. Laboratory examination reveals the following: hemoglobin, 5.1 g/dL; hematocrit, 15%; platelets, 12,000/μL; and white blood cell (WBC) count, 168,000/μL with 45% blast forms, 30% neutrophils, 20% lymphocytes, and 5% monocytes. Review of the peripheral blood smear confirms acute myeloid leukemia (M1 subtype, myeloblastic leukemia without maturation) with complex chromosomal abnormalities on cytogenetics. All of the following confer a poor prognosis for this patient EXCEPT:
A. Advanced age
B. Complex chromosomal abnormalities on cytogenetics
C. Hemoglobin below 7 g/dL
D. Prolonged interval between symptom onset and diagnosis
E. WBC count above 100,000/μL
III-86. The evaluation in a newly diagnosed case of acute lymphoid leukemia should routinely include all of the following EXCEPT:
A. Bone marrow biopsy
B. Cell-surface phenotyping
C. Cytogenetic testing
D. Lumbar puncture
E. Plasma viscosity
III-87. All of the following infectious agents have been associated with development of a lymphoid malignancy EXCEPT:
A. Helicobacter pylori
B. Hepatitis B
C. Hepatitis C
E. Human herpes virus 8 (HHV8)
III-88. A 64-year-old man with chronic lymphoid leukemia and chronic hepatitis C presents for his yearly follow-up. His white blood cell count is stable at 83,000/μL, but his hematocrit has dropped from 35% to 26%, and his platelet count also dropped from 178,000/μL to 69,000/μL. His initial evaluation should include all of the following EXCEPT:
A. AST, ALT, and prothrombin time
B. Bone marrow biopsy
C. Coombs test
D. Peripheral blood smear
E. Physical examination
III-89. During a routine visit, a 68-year-old woman complains of 3 months of fatigue, abdominal fullness, and bilateral axillary adenopathy. On physical examination, vital signs are normal, and she has bilateral palpable axillary and cervical adenopathy and an enlarged spleen. A complete blood count is notable for a white cell count of 88,000 with 99% lymphocytes. A peripheral smear is shown in Figure III-89. Which of the following is the most likely diagnosis?
FIGURE III-89 (see Color Atlas)
A. Acute lymphoblastic leukemia
B. Acute myelogenous leukemia
C. Chronic lymphocytic lymphoma
D. Hairy cell leukemia
III-90. Which of the following carries the best disease prognosis with appropriate treatment?
A. Burkitt’s lymphoma
B. Diffuse large B-cell lymphoma
C. Follicular lymphoma
D. Mantle cell lymphoma
E. Nodular sclerosing Hodgkin’s disease
III-91. A 27-year-old man seeks medical attention for enlarging nodules in his neck. He reports they are non-tender and have been growing for more than 1 month. At first he thought they were caused by a sore throat, but over the past 3 weeks he has felt well with no fever, chills, throat pain, or other associated symptoms. He notes a slightly diminished appetite but no weight loss. He works as a video game developer, does not smoke or use illicit drugs, and is sexually active with numerous female partners. He has never been tested for HIV. A lymph node biopsy is performed and is shown in Figure III-91. Which of the following is the most likely diagnosis?
FIGURE III-91 (see Color Atlas)
A. Burkitt’s lymphoma
B. Cat scratch disease
C. CMV infection
D. Hodgkin’s disease
E. Non-Hodgkin’s lymphoma
III-92. Which of the following is the most likely finding in a patient with a “dry” bone marrow aspiration?
A. Chronic myeloid leukemia
B. Hairy cell leukemia
C. Metastatic carcinoma infiltration
E. Normal bone marrow
III-93. All of the following statements are true regarding the criteria to diagnose hypereosinophilic syndrome EXCEPT:
A. Increased bone marrow eosinophils must be demonstrated.
B. It is not necessary to have increased circulating eosinophils.
C. Primary myeloid leukemia must be excluded.
D. Reactive eosinophilia (e.g., parasitic infection, allergy, collagen vascular disease) must be excluded.
E. There must be less than 20% myeloblasts in blood or bone marrow.
III-94. All of the following statements regarding mastocytosis are true EXCEPT:
A. Elevated serum tryptase suggests aggressive disease.
B. Eosinophilia is common.
C. It is often associated with myeloid neoplasm.
D. More than 90% of cases are confined to the skin.
E. Urticaria pigmentosa is the most common clinical manifestation.
III-95. A 58-year-old man is evaluated in the emergency department for sudden onset cough with yellow sputum production and dyspnea. Aside from systemic hypertension, he is otherwise healthy. His only medication is amlodipine. Chest radiograph shows a right upper lobe alveolar infiltrate, and laboratory test results are notable for a blood urea nitrogen of 53 mg/dL, creatinine of 2.8 mg/dL, calcium of 12.3 mg/dL, total protein of 9 g/dL, and albumin of 3.1 g/dL. Sputum culture grows Streptococcus pneumonia. Which of the following tests will confirm the underlying condition predisposing him to pneumococcal pneumonia?
A. Bone marrow biopsy
B. Computed tomography of the chest, abdomen, and pelvis with contrast
C. HIV antibody
D. Sweat chloride testing
E. Videoscopic swallow study
III-96. A 64-year-old African American man is evaluated in the hospital for congestive heart failure, renal failure, and polyneuropathy. Physical examination on admission was notable for these findings and raised waxy papules in the axilla and inguinal region. Admission laboratories showed a blood urea nitrogen of 90 mg/dL and a creatinine of 6.3 mg/dL. Total protein was 9.0 g/dL with an albumin of 3.2 g/dL. Hematocrit was 24%, and white blood cell and platelet counts were normal. Urinalysis was remarkable for 3+ proteinuria but no cellular casts. Further evaluation included an echocardiogram with a thickened left ventricle and preserved systolic function. Which of the following tests is most likely to diagnose the underlying condition?
A. Bone marrow biopsy
B. Electromyogram (EMG) with nerve conduction studies
C. Fat pad biopsy
D. Right heart catheterization
E. Renal ultrasonography
III-97. A 75-year-old man is hospitalized for treatment of a deep venous thrombosis. He had recently been discharged from the hospital about 2 months ago. At that time, he had been treated for community-acquired pneumonia complicated by acute respiratory failure requiring mechanical ventilation. He was hospitalized for 21 days at that time and had discharged from a rehabilitation 2 weeks ago. On the day before admission, he developed painful swelling of his left lower extremity. Lower extremity Doppler ultrasonography confirmed an occlusive thrombus of his deep femoral vein. After an initial bolus, he is started on a continuous infusion of unfractionated heparin at 1600 U/hr because he has end-stage renal disease on hemodialysis. His activated partial thromboplastin time is maintained in the therapeutic range. On day 5, it is noted that his platelets have fallen from 150,000/μL to 88,000/μL. What is the most appropriate action at this time?
A. Continue heparin infusion at the current dose and assess for anti-heparin/platelet factor 4 antibodies.
B. Stop all anticoagulation while awaiting results of anti-heparin/platelet factor 4 antibodies.
C. Stop heparin infusion and initiate argatroban.
D. Stop heparin infusion and initiate enoxaparin.
E. Stop heparin infusion and initiate lepirudin.
III-98. A 48-year-old woman is evaluated by her primary care physician for a complaint of gingival bleeding and easy bruising. She has noted the problem for about 2 months. Initially, she attributed it to aspirin that she was taking intermittently for headaches, but she stopped all aspirin and nonsteroidal anti-inflammatory drug use 6 weeks ago. Her only medical history is an automobile accident 12 years previously that caused a liver laceration. It required surgical repair, and she did receive several transfusions of red blood cells and platelets at that time. She currently takes no prescribed medications and otherwise feels well. On physical examination, she appears well and healthy. She has no jaundice or scleral icterus. Her cardiac and pulmonary examination results are normal. The abdominal examination shows a liver span of 12 cm to percussion, and the edge is palpable 1.5 cm below the right costal margin. The spleen tip is not palpable. There are petechiae present on her extremities and hard palate with a few small ecchy-moses on her extremities. A complete blood count shows a hemoglobin of 12.5 g/dL, hematocrit of 37.6%, white blood cell count of 8400/μL with a normal differential, and a platelet count of 7500/μL. What tests are indicated for the workup of this patient’s thrombocytopenia?
A. Antiplatelet antibodies
B. Bone marrow biopsy
C. Hepatitis C antibody
D. Human immunodeficiency antibody
E. C and D
F. All of the above
III-99. A 54-year-old woman presents acutely with alterations in mental status and fever. She was well until 4 days previously when she began to develop complaints of myalgia and fever. Her symptoms progressed rapidly, and today her husband noted her to be lethargic and unresponsive when he awakened. She has recently felt well otherwise. Her only current medication is atenolol 25 mg daily for hypertension. On physical examination, she is responsive only to sternal rub and does not vocalize. Her vital signs are blood pressure of 165/92 mm Hg, heart rate of 114 beats/min, temperature of 38.7°C (101.7°F), respiratory rate of 26 breaths/min, and oxygen saturation of 92% on room air. Her cardiac examination shows a regular tachycardia. Her lungs have bibasilar crackles. The abdominal examination is unremarkable. No hepatosplenomegaly is present. There are petechiae on the lower extremities. Her complete blood count has a hemoglobin of 8.8 g/dL, hematocrit of 26.4%, white blood cell count of 10.2/μL (89% polymorphonuclear cells, 10% lymphocytes, 1% monocytes), and a platelet count of 54,000/μL. A peripheral blood smear is shown in Figure III-99. Her basic metabolic panel has a sodium of 137 meq/L, potassium of 5.4 meq/L, chloride of 98 meq/L, bicarbonate of 18 meq/L, BUN of 89 mg/dL, and creatinine of 2.9 mg/dL. Which statement most correctly describes the pathogenesis of the patient’s condition?
FIGURE III-99 (see Color Atlas)
A. Development of autoantibodies to a metalloproteinase that cleaves von Willebrand factor
B. Development of autoantibodies to the heparin-platelet factor 4 complex
C. Direct endothelial toxicity initiated by an infectious agent
D. Inherited disorder of platelet granule formation
E. Inherited disorder of von Willebrand factor that precludes binding with factor VIII
III-100. What is the best initial treatment for this patient?
A. Acyclovir 10 mg/kg intravenously every 8 hours
B. Ceftriaxone 2 g intravenously daily plus vancomycin 1 g intravenously twice daily
D. Methylprednisolone 1 g intravenously
E. Plasma exchange
III-101. Which of the following statements regarding hemophilia A and B is TRUE?
A. Individuals with factor VIII deficiency have a more severe clinical course than those with factor IX deficiency.
B. Levels of factor VIII or IX need to be measured before administration of replacement therapy in patients presenting with acute bleeding to calculate the appropriate dose of factor.
C. Primary prophylaxis against bleeding is never indicated.
D. The goal level of factor VIII or IX is greater than 50% in the setting of large-volume bleeding episodes.
E. The life expectancy of individuals with hemophilia is about 50 years.
III-102. A 24-year-old man is admitted to the hospital with circulatory collapse in the setting of disseminated meningococcemia. He is currently intubated, sedated, and on mechanical ventilation. He has received over 6 L of intravenous saline in the past 6 hours but remains hypotensive, requiring treatment with norepinephrine and vasopressin at maximum doses. He is making less than 20 mL of urine each hour. Blood is noted to be oozing from all of IV sites. His endotracheal secretions are blood tinged. His laboratory studies show a white blood cell count of 24,300/μL (82% neutrophils, 15% bands, 3% lymphocytes), hemoglobin of 8.7 g/dL, hematocrit of 26.1%, and platelets of 19,000/μL. The international normalized ratio is 3.6, the activated partial thromboplastin time is 75 seconds, and fibrinogen is 42 mg/dL. The lactate dehydrogenase level is 580 U/L, and the haptoglobin is less than 10 mg/dL. The peripheral smear shows thrombocytopenia and schistocytes. All of the following treatments are indicated in this patient EXCEPT:
A. Ceftriaxone 2 g intravenously twice daily
C. Fresh-frozen plasma
III-103. All the following are vitamin K–dependent coagulation factors EXCEPT:
A. Factor X
B. Factor VII
C. Protein C
D. Protein S
E. Factor VIII
III-104. A 31-year-old man with hemophilia A is admitted with persistent gross hematuria. He denies recent trauma and any history of genitourinary pathology. The examination is unremarkable. Hematocrit is 28%. All the following are treatments for hemophilia A EXCEPT:
A. Desmopressin (DDAVP)
B. Fresh-frozen plasma
D. Recombinant factor VIII
III-105. All of the following statements regarding the lupus anticoagulant (LA) are true EXCEPT:
A. LAs typically prolong the activated partial thromboplastin time.
B. A 1:1 mixing study will not correct in the presence of LAs.
C. Bleeding episodes in patients with LAs may be severe and life threatening.
D. Female patients may experience recurrent midtrimester abortions.
E. LAs may occur in the absence of other signs of systemic lupus erythematosus.
III-106. All the following cause prolongation of the activated partial thromboplastin time that does not correct with a 1:1 mixture with pooled plasma EXCEPT:
A. Lupus anticoagulant
B. Factor VIII inhibitor
D. Factor VII inhibitor
E. Factor IX inhibitor
III-107. You are evaluating a 45-year-old man with an acute upper gastrointestinal (GI) bleed in the emergency department. He reports increasing abdominal girth over the past 3 months associated with fatigue and anorexia. He has not noticed any lower extremity edema. His medical history is significant for hemophilia A diagnosed as a child with recurrent elbow hemarthroses in the past. He has been receiving infusions of factor VIII for most of his life and received his last injection earlier that day. His blood pressure is 85/45 mm Hg with a heart rate of 115 beats/min. His abdominal examination is tense with a positive fluid wave. Hematocrit is 21%. Renal function and urinalysis are normal. His activated partial thromboplastin time is minimally prolonged, his international normalized ratio is 2.7, and platelets are normal. Which of the following is most likely to yield a diagnosis for the cause of his GI bleeding?
A. Factor VIII activity level
B. Helicobacter pylori antibody test
C. Hepatitis B surface antigen
D. Hepatitis C RNA
E. Mesenteric angiogram
III-108. You are managing a patient with suspected disseminated intravascular coagulopathy (DIC). The patient has end-stage liver disease awaiting liver transplantation and was recently in the intensive care unit with Escherichia coli bacterial peritonitis. You suspect DIC based on a new upper gastrointestinal bleed in the setting of oozing from venipuncture sites. Platelet count is 43,000/μL, international normalized ratio is 2.5, hemoglobin is 6 mg/dL, and D-dimer is elevated to 4.5. What is the best way to distinguish between new-onset DIC and chronic liver disease?
A. Blood culture
B. Elevated fibrinogen degradation products
C. Prolonged aPTT
D. Reduced platelet count
E. Serial laboratory analysis
III-109. All of the following genetic mutations are associated with an increased risk of deep venous thrombosis EXCEPT:
A. Factor V Leiden mutation
B. Glycoprotein 1b platelet receptor
C. Heterozygous protein C deficiency
D. Prothrombin 20210G
E. Tissue plasminogen activator
III-110. A 76-year-old man presents to an urgent care clinic with pain in his left leg for 4 days. He also describes swelling in his left ankle, which has made it difficult for him to ambulate. He is an active smoker and has a medical history remarkable for gastroesophageal reflux disease, deep venous thrombosis (DVT) 9 months ago that resolved, and well-controlled hypertension. Physical examination is revealing for 2+ edema in his left ankle. A D-dimer is ordered and is elevated. Which of the following makes D-dimer less predictive of DVT in this patient?
A. Age older than 70 years
B. History of active tobacco use
C. Lack of suggestive clinical symptoms
D. Negative Homan’s sign on examination
E. Previous DVT in the past year
III-111. A 22-year-old woman comes to the emergency department complaining of 12 hours of shortness of breath. The symptoms began toward the end of a long car ride home from college. She has no medical history, and her only medication is an oral contraceptive. She smokes occasionally, but the frequency has increased recently because of examinations. On physical examination, she is afebrile with respiratory rate of 22 breaths/min, blood pressure of 120/80 mm Hg, heart rate of 110 beats/min, and oxygen saturation on room air of 92%. The rest of her physical examination findings are normal. A chest radiograph and complete blood count are normal. Her serum pregnancy test result is negative. Which of the following is the indicated management strategy?
A. Check D-dimer and, if normal, discharge with non-steroidal anti-inflammatory therapy.
B. Check D-dimer and, if normal, obtain lower extremity ultrasound.
C. Check D-dimer and, if abnormal, treat for deep venous thrombosis/pulmonary embolism.
D. Check D-dimer and, if abnormal, obtain a contrast multislice computed tomography scan of the chest.
E. Obtain a contrast multislice computed tomography scan of the chest.
III-112. All of the anticoagulant or antiplatelet drugs listed are correctly matched with their mechanisms of action EXCEPT:
A. Abciximab—Glycoprotein IIb/IIIa receptor inhibitor
B. Clopidogrel—Adenosine diphosphate receptor blockade
C. Enoxaparin—Direct thrombin inhibition
D. Rivaroxaban—Factor Xa inhibition
E. Warfarin—Inhibition of production of the vitamin K–dependent clotting factors
III-113. A 66-year-old woman is prescribed clopidogrel and aspirin after implantation of a bare metal stent in her right coronary artery. Two weeks after the procedure, the woman presents to the emergency department with acute-onset chest pain and electrocardiographic changes consistent with an acute inferior myocardial infarction. Emergent cardiac catheterization confirms in-stent restenosis. The patient insists she has been adherent to her prescribed therapy. Which of the following statements most correctly described the most likely cause of the patient’s restenosis despite her current therapy?
A. She likely has aspirin resistance and should be treated with higher doses of aspirin to prevent a recurrence.
B. She likely has clopidogrel resistance caused by a genetic polymorphism of the CYP pathway.
C. She should have been treated with low-molecular-weight heparin to prevent this complication.
D. She should have been treated with warfarin to prevent this complication.
E. Because she has demonstrated resistance to clopidogrel, switching to prasugrel would not be useful to prevent further complications.
III-114. A 48-year-old woman is diagnosed with a deep venous thrombosis of her left lower extremity. When considering initial anticoagulant therapy, all of the following are advantages of low-molecular-weight heparins over heparin EXCEPT:
A. Better bioavailability
B. Dose-dependent clearance
C. Longer half-life after subcutaneous injection
D. Lower risk of heparin-induced thrombocytopenia
E. Predictable anticoagulant effect