Department of Medicine Grand Rounds Case Vignette
Benjamin F. Remo, M.D. April 11th, 2007
• CC: A 25-year-old female who presents with fatigue and fever for 2 weeks • HPI: The patient was in her usual state of good health until 2 weeks ago when she noted the sudden onset of low grade fever and fatigue with minimal exertion. The fever was constant and alleviated with acetaminophen. She has no known past medical history and does not take any prescribed medications. She denies any recent sick contacts, bleeding diathesis, or pain.
Physical Exam
• General: pale female appearing her stated age • T: 100.6 HR: 90 BP: 112/68 RR: 12 • HEENT: conjunctival pallor • Skin: warm erythematous left thigh
Labs
• • • • • • WBC 133 Hemoglobin 9.0 Platelets 76 PT/PTT wnl FDP negative Fibrinogen wnl
Hematocrit 27%
Peripheral Blood Smear
• Cluster of atypical promyelocytes
Preliminary Diagnosis
• Acute Promyelocytic Leukemia
Bone Marrow Aspirate
• Increased cellularity with infiltration of promyelocytelike blast cells
Therapeutic Course
• Immunophenotyping, cytochemistry, cytogenetics, and FISH were performed confirming Acute Promyelocytic Leukemia. • Induction phase treatment was initiated with all-trans retinoic acid (ATRA) and idarubicin • On day #2 of treatment, the patient developed fever, dyspnea, and bilateral pulmonary infiltrates: consistent with retinoic acid syndrome (RAS) • ATRA was stopped and dexamethasone instituted. Despite this, her respiratory condition worsened, requiring mechanical ventilation • Subsequent pulmonary infection with Candida spp, and the patient passed away on day #18 of induction therapy from respiratory failure.
Final Diagnosis
• Acute Promyelocytic Leukemia (AML with (15;17) (q22;q12); PML/RARa), intermediate typical/microgranular variant.
Clinical Question
• Which one of the following statement regarding acute promyelocytic leukemia is true? • A) Patient with APL has less tendency to develop DIC • B) Usually associated with translocation between chromosome 14 & 16 • C) Have over 80% remission and over 70% cure rate with treatment • D) Age <30 is a predictor of poor outcome
Answer
• Answer C • Induction therapy with ATRA plus anthracycline based chemotherapy produces remission rates of 80 to 95 percent in both previously untreated and relapsed patients. • Most treatment failures are due to early mortality (approximately 10 percent); more than one-half of these are hemorrhagic deaths due to thrombocytopenia and/or DIC. • Age <30 and initial WBC <10,000/microL are favorable prognostic factors for longer event-free survival.