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The New England Journal of MedicineAcute Promyelocytic Leukemia

Navigating the Complex Landscape of Acute Promyelocytic Leukemia: Insights and Management Strategies in a Challenging Case

A case study of acute promyelocytic leukemia stresses the critical importance of prompt recognition and treatment initiation in patients presenting with fever, weakness, and pancytopenia. This comprehensive analysis explores the clinical presentation, diagnostic approach, and management strategies employed in a complex case marked by differentiation syndrome, disseminated intravascular coagulation, and bacteremia, offering valuable insights for physicians encountering similar scenarios.

Key Points:

  • A 47-year-old man presented with fever, weakness, and left thigh swelling, leading to suspicion of an abscess, but further evaluation revealed pancytopenia and extreme elevation of the d-dimer level.
  • Peripheral-blood smear analysis showed promyelocytic blast cells with intracellular Auer rods, prompting concern for acute promyelocytic leukemia and sepsis, necessitating admission to the intensive care unit.
  • Induction chemotherapy with all-trans retinoic acid and prednisolone was initiated, followed by confirmation of acute promyelocytic leukemia based on genetic analysis for chromosomal translocation identifying a PML–RARA fusion gene.
  • Differentiation syndrome and disseminated intravascular coagulation complicated the hospital course, along with Staphylococcus aureus bacteremia and leg abscesses.
  • Molecular complete remission was achieved, leading to consolidation chemotherapy with arsenic trioxide and subsequent discharge on hospital day 72.

Acute promyelocytic leukemia (APL) develops in about 600 to 800 individuals each year in the United States, most often in adults around the age of 40 but also in children.

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