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INTRODUCTION

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CASE LEARNING OBJECTIVES

  • Recognize the major prognostic factors in childhood pre–B-cell acute lymphocytic leukemia

  • Identify the treatment-related complications associated with the therapy for acute lymphocytic leukemia

  • Identify the role of thiopurine methyltransferase metabolism with respect to 6-mercaptopurine toxicity

  • Develop a plan to evaluate the patient's current problems and determine if they are related to his ability to metabolize 6-mercaptopurine and select an appropriate new dose for this patient

  • Describe the late effects of chemotherapy related to the treatment of acute lymphocytic leukemia

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PATIENT PRESENTATION

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Chief Complaint (Mother's)

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"My son keeps having repeated episodes of infection and low blood counts. He needs to be transfused more often than the other patients I know who are on the same protocol."

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History of Present Illness

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MC is a 12-year-old Hispanic male who was diagnosed with pre–B-cell acute lymphoblastic leukemia (ALL) at age 11. His presenting WBC count was 7.5 × 103/mm3 (7.5 × 109/L) with 80% leukemic cells. He had no testicular disease and his cerebrospinal fluid showed no leukemic cells. Cytogenetic evaluation of his leukemic cells showed random chromosomal loss and gain. Tumor-cell ploidy showed a DNA content of 1.15. He had a prompt response to induction therapy with a morphologic remission at day 8. His end-of-induction minimal residual disease (MRD) was low. He recently completed his final "delayed intensification" treatment module and began "maintenance" treatment 5 months ago. Since the start of maintenance, he has had two admissions for fever of 101.3°F (38.5°C) and ANC of 250–300/mm3 (0.25–0.30 × 109/L), each lasting 7 days. Both episodes were associated with negative blood cultures. On each occasion, his maintenance chemotherapy [daily oral 6-mercaptopurine (6MP) and weekly oral methotrexate] was stopped until his ANC was above 500/mm3 (0.50 × 109/L), and he was afebrile for 48 hours.

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Past Medical History

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Full-term infant, uncomplicated birth, and neonatal history

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ALL s/p chemotherapy; history of 1 previous admission for fever and neutropenia during the delayed intensification phase of treatment. He has received 2 prior platelet transfusions, one each during induction and delayed intensification phases of treatment. He has received 3 prior RBC transfusions, the last of which was greater than 3 months ago

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Completely up-to-date on childhood immunizations for his age up to the time of his diagnosis for ALL

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Family History

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Noncontributory

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Social/Work History

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The patient is currently in seventh grade at an elementary school in a disadvantaged neighborhood where the first language of many residents is Spanish. He is a good student, and his parents were involved in his school and community in a positive way. There are two additional siblings in the home: a 6-year-old sister in kindergarten and a ...

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