MKSAP13-Hematology and Oncology

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<p>Hematology and Oncology:Question 1 A 67-year-old man in previously good health is hospitalized because of a 2-day history of fever and diminished consciousness. The patient responds inconsistently to verbal commands. His temperature is 39.5 C (103.1 F); he has tachycardia, and his blood pressure is 80/58 mm Hg. There is no bleeding. His hemoglobin is 12.1 g/dL, leukocyte count is 29,000/L with 80% neutrophils, and platelet count is 20,000/L. Which of the following studies should be obtained in this patient? A. Bone marrow aspiration and biopsy B. Factor VIII level C. Measurement of platelet-associated IgG D. Measurements of fibrin D-dimer and total fibrinogen E. Bleeding time Hematology and Oncology:Question 2 A 35-year-old woman has completed 6 months of anticoagulation therapy for pulmonary emboli. She had been using oral contraceptives for the past 10 years but stopped when the pulmonary emboli were detected. There is no family history of thrombosis. Two weeks after the discontinuation of warfarin, she undergoes coagulation studies. She is found to be heterozygous for the prothrom bin G2021 0A</p> <p>mutation. On the basis of her history and laboratory evaluation, what is the best recommendation for this patient? A. Resume oral anticoagulation therapy at a target INR of 2 to 3 B. Resume oral anticoagulation at a target INR of 1 .5 to 2 and advise her that it is safe to begin taking oral contraceptives again C. Advise the patient to take 81 mg of aspirin each day D. Prescribe a combination of aspirin and dipyridamole E. Advise the patient not to resume oral contraceptives and that additional therapy is not required for the thrombotic event Hematology and Oncology:Question 3 A 22-year-old man with sickle cell disease has four to six painful crises per year that require parenteral hydration and narcotics. Two weeks ago the patient developed an acute chest characterized by fever, dyspnea, and the radiographic appearance of pulmonary infiltrates. The patients condition rapidly responded to aggressive exchange transfusions and empiric broad-spectrum antibiotics; he has been asymptomatic for 1 week. Laboratory studies: Hemoglobin 10.4 g/dL</p> <p>Hematocrit 32% Leukocyte count 12,000/L Platelet count 277,000/L Hemoglobin electrophoresis shows the following results. Hemoglobin A (2 2) 62% (normal &gt; 97.5%) Hemoglobin A2(2 2) 2% (normal&lt; 2.5%) Hemoglobin F (22) 1% (normal 0%) Hemoglobin S (2 S2) 35% (normal 0%) What is the best next step in this patients management? A. Long-term transfusion therapy B. Hydroxyurea therapy C. Consultation for therapeutic gene therapy D. Consultation for therapeutic bone marrow transplantation E. Long-term oxygen therapy Hematology and Oncology:Question 4 A 24-year-old woman is evaluated in the emergency department because of fever, chills, sore throat, and difficulty talking. Two months ago she was diagnosed with schizophrenia, and clozapine therapy was initiated, at which time her complete blood count was normal. On physical examination, her tonsils are swollen and red bilaterally; she has submandibular lymphadenopathy and high</p> <p>fever. Her temperature is 40 C (104 F), pulse rate 140/min, and blood pressure is 90/50 mm Hg. Her hemoglobin is 12 g/dL, hematocrit is 36%, leukocyte count is 2000/L, and platelet count is 180,000/L. Throat and blood cultures are obtained; broadspectrum intravenous antibiotic therapy is initiated and clozapine is discontinued. Which of the following is the most appropriate next step for this patient? A. Bone marrow aspiration and biopsy B. Initiation of subcutaneous granulocyte colony-stimulating factor daily C. Granulocyte transfusion D. Search for a suitable bone marrow donor E. Test for HIV Hematology and Oncology:Question 5 A 46-year-old woman with alcoholism is evaluated because of a 4-day history of nausea and vomiting after a recent binge. She has diffuse epigastric pain. She has been hospitalized intermittently for years for medical complications related to alcohol abuse, including cirrhosis with ascites and bleeding esophageal varices. On physical examination, her temperature is 37.3 C (99.1 F), pulse</p> <p>rate is 68/min, and blood pressure is 135/70 mm Hg. She has jaundice and is icteric. Her lungs are clear to auscultation. The remainder of her physical examination is unremarkable. Laboratory studies: Hemoglobin 7.1 g/dL Leukocyte count 4500/L Platelet count 115,000/L Mean corpuscular volume 107 fL Reticulocyte count 5.4% Serum alkaline phosphatase 592 U/L Serum aspartate aminotransferase 198 U/L Serum alanine aminotransferase 166 U/L Serum total bilirubin 9.2 mg/dL Serum indirect bilirubin6.4 mg/dL Serum albumin 2.2 g/dL Serum folate 12 ng/mL Which of the following diagnoses best explains the findings? A. Spur cell anemia B. Folic acid deficiency C. Hypersplenism D. Acanthocytosis Hematology and Oncology:Question 6 A 62-year-old man is evaluated because of increasing fatigability. He previously felt fine. He has recently been found to have</p> <p>osteomyelitis underlying a diabetic foot ulcer and is being treated with ciprofloxacin and amoxicillin/clavulanate. Laboratory studies: Hemoglobin 9.8 g/dL Mean corpuscular volume 79 fL Serum creatinine0.9 mg/dL Serum iron 38 g/dL Serum total iron-binding capacity 190 g/dL Serum ferritin 298 ng/mL Which of the following constitutes appropriate additional therapy? A. Cyanocobalamin B. Ferrous sulfate C. Parenteral iron D. Recombinant human erythropoietin and ferrous sulfate Hematology and Oncology:Question 7 A previously healthy, 59-kg (130-Ib), 35-yearold woman delivers her fifth child after an uncomplicated pregnancy and labor. Her blood type is group O, Rh-positive. Soon after the birth, vaginal bleeding is noted that requires emergency transfusion of four units of packed red blood cells. Results of an antibody screen and direct antiglobulin test were negative at 2 months gestation and again when the patient was hospitalized for the delivery. Seven days</p> <p>later, faint scleral icterus is noted. The hemoglobin is 9.5 g/dL (approximately 1 g/dL less than at discharge after delivery). Serum total bilirubin is 2.1 mg/dL, with a direct component of 0.3 mg/dL. Serum lactate dehydrogenase level is 200 U/L, and haptoglobin is 20 mg/dL. Direct antiglobulin (Coombs) testing shows IgG coating the erythrocytes. There is no hemoglobinemia, and the urine is negative for blood. What is the most likely cause of hemolysis in this patient? A. Improper storage of the units of blood prior to transfusion B. Autoimmune hemolytic anemia C. Glucose-6-phosphate dehydrogenase deficiency D. Erroneous transfusion of group A blood E. Hemolytic transfusion reaction Hematology and Oncology:Question 8 A 60-year-old woman is evaluated because of a 1-year history of fatigue. Her menses ended when she was 50 years old, and she has not had any blood loss since that time. She also notes that she is short of breath with exertion. She recalls that a bone marrow biopsy was done some years ago. She is not sure about her family history because her father died</p> <p>when she was young and her mother died of liver complications at an early age. On physical examination, she has a bronze hue to her skin. The liver is enlarged 3 cm below the right costal margin. She has swelling of the first and second metacarpophalangeal joints of both hands. Laboratory studies: Hemoglobin 13.0 g/dL Hematocrit 40% Serum ferritin 1900 ng/mL Transferrin saturation 70% Fasting plasma glucose 115 mg/dL Serum alanine aminotransferase 80 U/L Serum aspartate aminotransferase 35 U/L A copy of the bone marrow study that she mentioned reveals only trace macrophage iron stores. A polymerase chain reaction test of mutations in the HFE gene is performed on the basis of the clinical picture, and the patient is found to be a homozygote for the C282Y mutation. Which of the following is the next best step in this patients management? A. Liver biopsy B. Phlebotomy C. Follow-up of liver function and iron status D. Liver biopsy and phlebotomy</p> <p>Hematology and Oncology:Question 9 A 37-year-old man is hospitalized because of fever and right-sided chest pain. He has been having fatigue and recently developed dyspnea on exertion and intermittent chills. At the age of 29 years he was diagnosed with stage III Hodgkins disease and treated with multiagent chemotherapy and radiation therapy. A year ago, he developed mild anemia with no obvious cause. He takes thyroid hormone replacement when he remembers and has used fexofenadine as needed for allergic rhinitis for the past 5 years. On physical examination, his temperature is 38.7 C (101.7 F), pulse rate is 112/min, and blood pressure is 110/70 mmHg. There is dullness at the right lower lung and egophony. The hemoglobin is 8.5 g/dL, hematocrit is 26 %, leukocyte count is 2200/L, and platelet count is 70,000/L. What is the most likely diagnosis? A. Relapsed Hodgkins disease B. Sepsis C. Hypothyroidism D. Secondary myelodysplastic syndrome E. Drug-induced bone marrow suppression Hematology and Oncology:Question 10 A 43-year-old man with severe acquired</p> <p>aplastic anemia has not responded to immunosuppressive agents. He remains neutropenic and transfusion-dependent for platelets and red cells. He has an HLA-identical brother who has been cleared as a donor for his planned allogeneic stem cell transplant. They are both cytomegalovirus-seronegative. Which of the following would be prevented by using irradiated cellular blood products for this patient? A. Cytomegalovirus disease B. Alloimmunization C. Transfusion-related graft-versus-host disease D. Febrile nonhemolytic transfusion reaction E. Hemolytic transfusion reaction Hematology and Oncology:Question 11 A 40-year-old Chinese woman is evaluated because of pulmonary emboli that developed 1 week after she arrived in the United States after a 16-hour airplane trip. She has no other medical problems. She had taken oral contraceptives for several years and had one uncomplicated pregnancy but is not taking any medications at present. Her older brother and father both have a history of thrombosis. Results of a complete blood count, prothrombin time, and activated partial thromboplastin time</p> <p>are normal. Which of the following studies should be done at this time? A. Factor V Leiden and prothrombin G20210A mutation analyses B. Protein C, protein 5, and antithrombin III measurements C. Testing for a lupus anticoagulant D. Screening tests for cancer including CT scans of the chest, abdomen, and pelvis E. No testing is required Hematology and Oncology:Question 12 A 34-year-old woman is evaluated because of progressive fatigue and recurrent attacks of abdominal pain. She weighs 61.2 kg (135 Ib). Her pulse rate is 110/min and her blood pressure is 110/70 mm Hg. She is pale, and her spleen is palpable 4 cm below the costal margin. Laboratory studies: Hemoglobin 6 g/dL Hematocrit 20% Leukocyte count 2500/L Platelet count 80,000/L Reticulocyte count 10% Blood smear Anisocytosis and polychromatophilia Haptoglobin 0 mg/dL</p> <p>Serum lactate dehydrogenase 645 U/L There is high level of hemosiderin in the urine. What is the most likely cause of this patients pancytopenia? A. Autoimmune hemolytic anemia B. Aplastic anemia C. Myelodysplastic syndrome D. Paroxysmal nocturnal hemoglobinuria E. Acute myeloid leukemia Hematology and Oncology:Question 13 A 45-year-old man with alcoholic cirrhosis is evaluated because of hematemesis from active bleeding due to esophageal varices. He receives eight units of uncrossmatched group O packed cells over 3 hours, but his blood pressure remains unstable and his bleeding continues. When he was first evaluated in the emergency department, his prothrombin time and partial thromboplastin time were 34 s and 57 s, respectively. The blood bank never received a specimen for type and screen, and no pretransfusion blood is available for testing. What is the most appropriate transfusion product to treat this patients bleeding? A. Group O whole blood B. Group AB whole blood</p> <p>C. Group O fresh frozen plasma D. Group AB fresh frozen plasma Hematology and Oncology:Question 14 A 24-year-old man who moved to this country from Sweden 15 years ago is evaluated because of severe weakness and shortness of breath. He has a 4-year history of HIV infection and has had intermittent infections, predominantly sinusitis, over the past year. Two months ago, his hemoglobin was 10.8 g/dL, leukocyte count was 3900/L, and platelet count was 220,000/L. He has been noncompliant with his antiretoviral medications because of the side effects, but admits to taking trimethoprim-sulfamethoxazole twice daily for the past week. His appetite is poor. Cn physical examination, he exhibits extreme pallor. His temperature is 37.5 C (99.5 F), his pulse rate is 110/min, and his blood pressure is 130/89 mm Hg. His respiratory rate is 22/min at rest, increasing to 33/min when walking. He has no skin lesions. His conjunctivae are pale. He has generalized shotty lymphadenopathy. Chest radiography is normal. The remainder of his physical examination is unremarkable. Laboratory studies:</p> <p>Hemoglobin 4 g/dL Leukocyte count 2400/L Platelet count 129,000/L Mean corpuscular volume 101 fL Reticulocyte count 0% Peripheral blood smear No abnormalities Serum total bilirubin 3.2 mg/dL Serum indirect bilirubin2.8 mgldL Serum vitamin B12 222 pg/mL Serum folate 14 ng/mL What is the most likely diagnosis? A. Drug-induced marrow suppression B. Parvoviral infection C. HIV-induced marrow dysplasia D. Autoimmune hemolytic anemia Hematology and Oncology:Question 15 A 24-year-old black woman is evaluated at an urgent care clinic because of sore throat, temperature of 39.4 C (103 F), chills, and severe fatigue of several weeks duration. She does not take any prescription or over-thecounter medications and she has never been ill. She lives with her two sisters. On physical examination she has pallor and swollen and red tonsils bilaterally. Her pulse rate is 120/min, and her blood pressure is 90/50 mm Hg. Her hemoglobin is 6 g/dL, leukocyte count 100/L, platelet count</p> <p>17,000/L, and reticulocyte count 0%. After throat and blood cultures are obtained, broad-spectrum intravenous antibiotic therapy is administered, and the patient receives a transfusion of cytomegalovirus-negative irradiated red blood cells. Bone marrow aspirate and biopsy show scattered plasma cells and almost complete absence of myeloid elements. What is the most appropriate therapy for longterm survival of this patient? A. Intravenous immunoglobulin, 500 mg/kg daily for 4 days B. Allogeneic stem cell transplantation from an HLA-identical sister C. Oral melphalan and prednisone D. Antithymocyte globulin and cyclosporine E. Long-term transfusion and iron chelation therapy Hematology and Oncology:Question 16 A 63.5-kg (140-Ib) 29-year-old woman underwent repeat thoracic spinal fusion surgery. She had extensive bleeding and received a total of 20 units of packed red blood cells. The surgery lasted 3 hour...</p>