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1 | |
Which of the following statements concerning the musculoskeletal abnormalities in patients with sickle cell disease is correct? |
| | A) | Hand-foot syndrome characterized by diffuse swelling, tenderness, and warmth of the hands and feet in children under the age of 5 leads to chronic disability in about 10% of patients. |
| | B) | Acute arthritis seen in patients with sickle cell crisis is often associated with high white cell counts in joint effusions. |
| | C) | The bone pain in sickle cell crisis is due to bone and bone marrow infarction. |
| | D) | Acute gouty arthritis is common in patients with sickle cell disease. |
| | E) | Total hip replacement is contraindicated in patients with sickle cell anemia who sustain avascular necrosis of the femoral head. |
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2 | |
A 58-year-old man presents with fatigue. His physical examination is normal except for the presence of splenomegaly. CBC discloses hematocrit, 29%; platelet count, 90,000/ìL; WBC, 2700/ìL; and an essentially normal red cell morphology (differential 12% monocytes, 12% granulocytes, and 76% lymphocytes). A bone marrow aspirate and biopsy were performed. The aspirate was dry and the biopsy is pending. Based on the available information, the most likely diagnosis in this case is |
| | A) | chronic lymphocytic leukemia (CLL) |
| | B) | hairy cell leukemia |
| | C) | chronic myeloid leukemia (CML) |
| | D) | myelofibrosis |
| | E) | multiple myeloma |
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3 | |
A 58-year-old chronic alcoholic and heavy smoker presents with a 3-cm, firm, right midcervical neck mass. An excisional biopsy reveals squamous cell carcinoma. Which of the following is the most appropriate approach at this time? |
| | A) | Bronchoscopy, esophagoscopy, and laryngoscopy |
| | B) | CT of the neck |
| | C) | CT of the brain |
| | D) | Neck dissection |
| | E) | Radiation therapy |
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4 | |
A 28-year-old man with newly diagnosed acute myelogenous leukemia spikes a temperature to 38.7°C (101.7°F) on the sixth day of induction therapy. He feels well and has no physical complaints. His only medicine is intravenous cytosine arabinoside, 140 mg every 12 h. Physical examination is unrevealing. His white blood count is 900/L, of which 10% are granulocytes and the rest mostly lymphocytes; platelet count is 24,000/L. Findings on chest x-ray and urinalysis are normal.
After obtaining appropriate cultures, the man's physician should |
| | A) | observe closely for the development of a clinically evident source of fever |
| | B) | begin antibiotic therapy with gentamicin and mezlocillin |
| | C) | begin granulocyte transfusion and antibiotic therapy with gentamicin and mezlocillin |
| | D) | begin gammaglobulin treatment and antibiotic therapy with gentamicin and mezlocillin |
| | E) | begin antibiotic therapy with amphotericin, gentamicin, and mezlocillin |
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5 | |
Coumarin-induced skin necrosis is occasionally associated with the institution of oral anticoagulants in patients with |
| | A) | antithrombin III deficiency |
| | B) | protein C deficiency |
| | C) | factor VIII deficiency |
| | D) | plasminogen deficiency |
| | E) | dysfibrinogenemias |
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6 | |
A 55-year-old woman presents to the emergency department because her family notes that she has yellow skin. The patient has lost 7 kg (15 lbs) over the past 3 months but states that this is because she has been dieting in preparation for her daughter's wedding. Her past medical history is significant only for vitiligo. Her physical examination is unremarkable except for the presence of scleral icterus and a yellow tinge to the skin. Laboratory evaluation reveals hematocrit of 17%, WBC count of 2500/L, and platelet count of 70,000/L. Serum chemistries are normal except for direct bilirubin of 51 mol/L (3 mg/dL) and indirect bilirubin of 12 mol/L (0.7 mg/dL). The patient's reticulocyte count is 3%. MCV is 108 fL. Which one of the following additional laboratory findings would most likely be associated with this patient's clinical syndrome? |
| | A) | Clonal chromosomal abnormalities on karyotypic analysis of the bone marrow |
| | B) | Positive direct Coombs' test |
| | C) | Extrahepatic biliary obstruction |
| | D) | Decreased gastric fluid pH |
| | E) | Antiparietal cell antibody |
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7 | |
A 43-year-old woman was diagnosed as having an infiltrating ductal carcinoma of the right breast when she was 41. The lesion was 3 cm in diameter. Lymph node examination was negative. Estrogen and progesterone receptors were negative. She underwent a lumpectomy, followed by radiation therapy to the breast and chest wall, followed by six cycles of cyclophosphamide plus doxorubicin chemotherapy. At this time she feels well but has two 2-cm nodules in each lung on chest radiography. These were not present on prior chest radiographs. In discussing the situation with the patient, which of the following statements is correct? |
| | A) | These nodules are a late manifestation of cyclophosphamide toxicity. |
| | B) | Tamoxifen therapy should be employed at this time. |
| | C) | Autologous bone marrow transplantation offers this patient the best chance for long-term disease- free survival. |
| | D) | An antibody to the erbB2 proto-oncogene may improve the response to chemotherapy. |
| | E) | The patient has a median survival of approximately 6 to 12 months. |
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8 | |
A 27-year-old woman presents with stage II (breast and lymph node involvement) right breast cancer. Her family history is markedly positive for other tumors. One of her sisters developed an osteogenic sarcoma at age 17, her brother was diagnosed with acute leukemia at age 5, her mother died of breast cancer, and she has two uncles with soft-tissue sarcomas, both developing this disease when in their thirties. This patient's peripheral blood lymphocytes would be most likely to reveal which of the following abnormalities? |
| | A) | Retinoblastoma gene mutation |
| | B) | p53 gene mutation |
| | C) | Translocation between chromosomes 9 and 22 |
| | D) | Translocation between chromosomes 8 and 14 |
| | E) | Mutations of epidermal growth factor receptor gene |
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9 | |
A patient with a myelodysplastic syndrome (subtype, refractory anemia with ringed sideroblasts) has been transfusion-dependent for the past 2 years. The patient has received a total of 50 units of packed red blood cells. His physical examination is normal except for hyperpigmentation. Laboratory evaluation reveals mild glucose intolerance. A trial of erythropoietin was unsuccessful. Which of the following would be the most important therapeutic approach at this time? |
| | A) | Granulocyte colony-stimulating factor (G-CSF) |
| | B) | Phlebotomy |
| | C) | Ascorbic acid |
| | D) | Desferrioxamine |
| | E) | Hypertransfusion (maintain hematocrit at 40%) |
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10 | |
A 26-year-old woman has painful mouth ulcers. Six weeks ago, she was started on propylthiouracil for hyperthyroidism. She is afebrile, and physical examination is unremarkable except for several small oral aphthous ulcers. White blood cell count is 200/L (15% neutrophils, 80% lymphocytes, 5% monocytes); hemoglobin concentration, hematocrit, and platelet count are normal. The woman's physician should stop the prophylthiouracil and |
| | A) | schedule a follow-up outpatient appointment |
| | B) | arrange for HLA typing of her siblings in preparation for bone marrow transplantation |
| | C) | prescribe oral prednisone, 1 mg/kg |
| | D) | hospitalize her for broad-spectrum antibiotic therapy |
| | E) | hospitalize her for white blood cell transfusion |
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11 | |
A 30-year-old black woman with long-standing sickle cell anemia presents with severe pain in the chest and abdomen ~1 week after having an upper respiratory infection. No intrathoracic or intraabdominal pathology was immediately obvious on routine physical examination and laboratory evaluation. The most appropriate therapeutic intervention at this point is |
| | A) | hypertransfusion |
| | B) | hydration and narcotic analgesia |
| | C) | hydroxyurea |
| | D) | broad-spectrum antibiotics |
| | E) | exploratory laparotomy |
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12 | |
In persons who have chronic myelogenous leukemia, the translocation that accounts for the Philadelphia chromosome most commonly is found in |
| | A) | all cells of the body |
| | B) | all three hematopoietic cell lines but not in nonhematopoietic cells |
| | C) | all cells of the granulocytic cell line but not in nongranulocytic cells |
| | D) | all bone marrow stem cells but not in mature cells |
| | E) | all bone marrow stem cells and certain mature granulocytes |
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13 | |
A 45-year-old man presents with fatigue. Two years ago the patient received six cycles of combination chemotherapy (each cycle consisted of cyclophosphamide, doxorubicin, vincristine, and prednisone) for non-Hodgkin's lymphoma in chest and abdominal sites. The patient entered complete remission and has been followed expectantly since that point. His last prior visit was 3 months ago at which time he had no evidence of recurrent lymphoma, felt well, and had a normal laboratory examination. At this time his physical examination is remarkable for a purple discoloration of the fingertips, ears, and nose. The patient is somewhat pale. There is no evidence for peripheral lymphadenopathy. Laboratory studies include the following: white count 10,000/L (differential 60% neutrophils, 10% bands, 10% lymphocytes, 10% monocytes, 3% eosinophils, 1% basophils, 2% metamyelocytes, 1% myelocytes, and 1% nucleated red blood cell), hematocrit 28% and platelet count 300,000/L. The following results are also found: MCV 98 fL, lactic dehydrogenase 6.8 kat/L (400 U/L), total bilirubin 51 mol/L (3.0 mg/dL), and direct bilirubin 5.1 mol/L (0.3 mg/dL). Review of the peripheral blood smear reveals clumped red cells. A routine direct Coombs' test is negative. Additional laboratory testing would most likely reveal |
| | A) | positive direct Coombs' test (using anti-IgG antisera) if specimen is processed without allowing cooling |
| | B) | positive indirect Coombs' test detected with anti- IgG antibodies |
| | C) | circulating antibodies against Epstein-Barr virus |
| | D) | circulating antibodies against fetal red blood cells |
| | E) | circulating antibodies against Mycoplasma pneumoniae |
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14 | |
A 70-year-old man presents with back pain and fatigue. Workup reveals hematocrit of 30%, white blood cell count of 3.5/mL, and platelet count of 220,000/mL. Serum calcium and serum creatinine are normal. However, serum protein electrophoresis reveals a monoclonal protein of 42 g/L (4.2 g/dL), proved to be IgG-? based on immunoelectrophoresis. Bone marrow examination discloses sheets of dysplastic plasma cells, and skeletal survey reveals multiple osteolytic legions in the skull and spine. The patient is started on melphalan, prednisone, and erythropoietin. Which of the following would be most reasonable to add to the patient's therapeutic regimen at this time? |
| | A) | G-CSF |
| | B) | Anti-interleukin (IL)6 antibodies |
| | C) | Pamidronate |
| | D) | Fludarabine |
| | E) | Doxorubicin |
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15 | |
A 25-year-old, previously healthy woman presents with jaundice, confusion, and fever. Initial physical examination is unremarkable except for scattered petechiae on the lower extremities, scleral icterus, and disorientation on mental status examination. Laboratory examination discloses the following: hematocrit, 27%; white cell count, 12,000/L; platelet count, 10,000/L; bilirubin, 85 mol/L (5 mg/dL); direct bilirubin, 10 mol/L (0.6 mg/dL); urea nitrogen, 21 mmol/L (60 mg/dL); creatinine, 400 mol/L (4.5 mg/dL). Red blood cell smear discloses fragmented red blood cells and nucleated red blood cells. Prothrombin, thrombin, and partial thromboplastin times are all normal.
The most effective and appropriate therapeutic maneuver is likely to be |
| | A) | plasmapheresis |
| | B) | administration of aspirin |
| | C) | administration of high-dose glucocorticoids |
| | D) | administration of high-dose glucocorticoids plus cyclophosphamide |
| | E) | splenectomy |
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16 | |
A 38-year-old woman presents with redness and burning in the distal extremities. She has no other complaints. She has never been pregnant. Physical examination is normal except for redness of the fingertips and splenomegaly. Laboratory examination reveals hematocrit 40%, WBC count 9000 with a normal differential, and platelet count of 950,000/L. Other laboratory studies include reticulocyte count of 1%, bone marrow examination that discloses a hypercellular marrow with megakaryocytic hyperplasia and hyperlobated megakaryocytes, absent collagen deposition, and the presence of normal amounts of bone marrow iron. Cytogenetic studies reveal a normal female karyotype. A red cell mass study is normal. Which of the following statements concerning the patient's condition is true? |
| | A) | Observation is indicated. |
| | B) | Splenectomy should be performed. |
| | C) | Oral administration of chlorambucil, 0.4 mg/kg daily for 5 days, should begin. |
| | D) | Aspirin, 2 tablets every 6 h, should be administered. |
| | E) | Hydroxyurea, 1000 mg daily orally, is indicated. |
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17 | |
A 42-year-old woman presents with epistaxis and gum bleeding. Physical examination is remarkable for a temperature of 38°C (100.4°F) and petechiae on the lower extremities. Laboratory evaluation includes a hematocrit of 29%, platelet count of 15,000/L, and WBC of 2100/L (differential including 22% blasts, 30% promyelocytes, 20% lymphocytes, 10% monocytes, 2% myelocytes, and 3% metamyelocytes). PT is 15 s and PTT is 55 s. Bone marrow examination discloses a hypercellular marrow infiltrated with myeloblasts and heavily granulated promyelocytes. Myeloperoxidase stain of a bone marrow aspirate smear is markedly positive and demonstrates numerous intracellular rodlike forms. The patient is begun on all-trans retinoic acid. Which of the following is the most likely complication of this therapy? |
| | A) | Worsening of disseminated intravascular coagulopathy |
| | B) | Infection during neutropenia |
| | C) | Respiratory distress |
| | D) | Uric acid nephropathy |
| | E) | Mucositis |
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18 | |
Which of the following statements regarding toxic effects of chemotherapy is correct? |
| | A) | Anthracyclines are relatively nonmyelosuppressive. |
| | B) | Vincristine is a relatively weak myelosuppressive agent and can be administered during periods of low blood counts. |
| | C) | Cisplatin-induced nausea and vomiting are mild. |
| | D) | The use of bleomycin has been associated with secondary leukemia. |
| | E) | Cisplatin can produce hypercalcemia by imparing renal excretion. |
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19 | |
A 45-year-old man develops leukocytosis and fatigue. Workup reveals infiltration of the bone marrow with lymphoblasts. A sample of bone marrow is also sent for immunologic and cytogenetic analysis. Which of the following findings would be associated with the best prognosis? |
| | A) | Common acute lymphocytic leukemia antigen (CALLA) CD10 positivity, normal cytogenetics |
| | B) | CALLA CD10 positivity, t(9;22) |
| | C) | Surface immunoglobulin positivity, t(8;14) |
| | D) | My10 (CD34) positivity, normal cytogenetics |
| | E) | My7 (CD13) positivity, t(4;11) translocation |
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20 | |
Two years ago a 68-year-old man was found to have a prostate nodule on routine examination. Biopsy revealed poorly differentiated prostatic adenocarcinoma; staging studies failed to reveal any evidence of extraprostatic spread. Because of a desire to maintain potency, the patient opted for radiation therapy as primary treatment. Except for requiring lower extremity revascularization for intractable claudication, he did well until recently, when he developed pain in his right hip. Prostate specific antigen was elevated. Bone scan revealed areas of positive uptake in the pelvis and ribs (not present on the original staging study). The patient expresses a desire not to have a bilateral orchiectomy, "unless it would significantly improve my quality of life or survival compared with other therapies."
The most appropriate strategy at this point is to |
| | A) | biopsy one of the bony lesions |
| | B) | administer cisplatin and 5-fluorouracil |
| | C) | administer leuprolide and flutamide |
| | D) | administer diethylstilbestrol (DES) at low dose |
| | E) | perform an orchiectomy |
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21 | |
A 72-year-old man with known benign prostatic hypertrophy develops fever and flank pain. He rapidly becomes very ill. He presents to the emergency room with a blood pressure of 80/40 mmHg, heart rate of 120, and a temperature of 39.5°C (103°F). His urine shows numerous white cells. His laboratory examination is remarkable for a white count of 2000, hematocrit of 28%, and platelet count of 10,000. The PT and PTT are elevated. The most appropriate way to deal with this patient's coagulopathy is |
| | A) | intravenous -aminocaproic acid |
| | B) | intravenous heparin |
| | C) | platelets and fresh-frozen plasma |
| | D) | antithrombin 3 concentrates |
| | E) | intravenous antibiotics |
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22 | |
A 65-year-old woman presents with bleeding gums. Except for the presence of petechiae on the ankles, the physical examination is unremarkable. The white blood cell count is 500/L with 10% neutrophils, the hematocrit is 25%, and the platelet count is 10,000/L. Examination of a bone marrow biopsy reveals a cellularity of 10%. The few cells available for scrutiny do not reveal any morphologic abnormalities. The patient has no history of recent drug ingestion or hepatitis and has never experienced any similar problems. The most appropriate therapy for this patient would be |
| | A) | prednisone |
| | B) | low-dose ara-C |
| | C) | plasmapheresis |
| | D) | antithymocyte globulin, cyclosporine, methylprednisone, and G-CSF |
| | E) | G-CSF, erythropoietin, and IL-11 |
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23 | |
A patient being treated for refractory anemia has required monthly transfusions of 2 units of packed red blood cells over the past several months. Three days after receiving 2 units of packed red blood cells for a hematocrit of 22%, the patient's hematocrit was 27%. One week after the transfusion the hematocrit is 22%; the patient feels ill, has a low-grade fever, and is mildly jaundiced. Which of the following statements about this situation is incorrect? |
| | A) | This problem is probably due to autoantibodies. |
| | B) | Intravascular hemolysis has probably occurred. |
| | C) | The Rh status of donor and recipient should be rechecked. |
| | D) | If the patient is Rh-positive, one should look for anti-Kell or anti-Duffy antibodies in the patient's serum. |
| | E) | A positive direct Coombs' test is unlikely. |
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24 | |
Intravenous aprotinin is given to patients undergoing cardiac surgery to reduce blood loss. The mechanism of action is |
| | A) | primary inhibition of fibrinolysis (prevents dissolution of formed clots) |
| | B) | prevention of activation of the clotting cascade |
| | C) | prevention of activation of the clotting cascade and inhibition of fibrinolysis |
| | D) | enhancement of platelet activity |
| | E) | enhancement of platelet activity and inhibition of activation of the clotting cascade |
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25 | |
Which of the following statements regarding ovarian cancer is correct? |
| | A) | A surgical debulking procedure is unhelpful. |
| | B) | Nulliparity is a risk factor. |
| | C) | A history of cervical cancer is a risk factor. |
| | D) | (D) Stromal cell and germ cell tumors of the ovary are the most common histologic subtypes. |
| | E) | Histologic grade is not an important prognostic factor. |
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26 | |
A 65-year-old woman on hemodialysis for chronic renal failure requires an urgent dental extraction for an abscessed tooth. Of the following, the most appropriate agent to administer to reduce the risk of significant bleeding would be |
| | A) | desmopressin |
| | B) | conjugated estrogen |
| | C) | erythropoietin |
| | D) | fresh-frozen plasma |
| | E) | -Aminocaproic acid |
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