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Self-Assessment Questions

Hematology

Chronic Myelogenous Leukemia and
Other Myeloproliferative Disorders: Review Questions


Jeffrey A. Zonder, MD

Dr. Zonder is an assistant professor of medicine, Karmanos Cancer Institute,
Wayne State University School of Medicine, Detroit, MI.



Choose the single best answer for each question.

Questions 1 and 2 refer to the following case study.

A 37-year-old woman presents to her physician with complaints of weight loss, night sweats, and left upper quadrant abdominal pain. On examination, she appears thin and pale and has mild tachycardia, no lymphadenopathy, and a tender spleen tip palpable 8 cm below the costal margin. Results of laboratory tests reveal a leukocyte count of 120 × 103/µL, with a marked left shift and basophilia. Her hemoglobin level is 9.5 g/dL, and her platelet count is decreased at 97 × 103/µL. Chronic myelogenous leukemia (CML) is suspected.

1. Which of the following tests would be most helpful in confirming this patient’s diagnosis and determining the optimal initial treatment?

  1. Bone marrow biopsy and aspirate with cytogenetics
  2. Peripheral blood cytogenetics
  3. Peripheral blood fluorescence in situ hybridization (FISH)
  4. Peripheral blood neutrophil alkaline phosphatase staining
  5. Peripheral blood polymerase chain reaction (PCR)
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2. Testing confirms a diagnosis of CML. The patient is referred to a bone marrow transplant center, where human leukocyte antigen-typing of the patient and her 3 siblings is immediately initiated. In the meantime, the patient should begin which of the following therapies?

  1. Acute myelogenous leukemia (AML) induction chemotherapy
  2. Hydroxyurea
  3. Imatinib mesylate
  4. Interferon-alpa
  5. Supportive care with transfusions of packed erythrocytes and random donor platelets only
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3. A 75-year-old man with a history of hypercholesterolemia and type 2 diabetes develops transient speech problems and right arm weakness; however, these symptoms resolve en route to the hospital. On examination, his vital signs are normal and his spleen is markedly enlarged. His erythrocyte count is slightly elevated, and his platelet count is 1150 × 103/µL. Increased numbers of megakaryocytes and normal cytogenetics on a bone marrow biopsy confirm a diagnosis of essential thrombocythemia (ET). What is the best initial treatment for this patient?
  1. Interferon-alpa
  2. Hydroxyurea and aspirin
  3. Anagrelide and aspirin
  4. Imatinib mesylate
  5. Aspirin only
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4. A previously healthy, active 55-year-old man presents with complaints of a mild headache and pruritus that occurs after bathing. He appears flushed and has mild splenomegaly. His hemoglobin level is 22.2 g/dL, and his platelet count is 540 × 103/µL. Results of other laboratory tests are normal. Further testing confirms the diagnosis of polycythemia rubra vera. What is the optimal initial therapy for this patient?

  1. Hydroxyurea and low-dose aspirin
  2. Phlebotomy
  3. Phlebotomy and high-dose aspirin
  4. Phlebotomy and low-dose aspirin
  5. High-dose aspirin alone
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