A 13-year-old Hispanic girl is found to have a WBC count of 6,500/mm3 with 40% Auer rod–containing granular blasts that, by flow cytometry, express very bright CD33 but are negative for human leukocyte antigen–DR isotype (HLA-DR). She is oozing blood around her peripheral IV site. Coagulation studies reveal an international normalized ratio (INR) of 3.4, a fibrinogen of 170, and a markedly elevated D-dimer. Marrow aspirate shows nearly 90% blasts with a similar morphology. You send the marrow to the fluorescence in situ hybridization (FISH) lab and request STAT testing for the most likely recurrent genetic abnormality based on the clinical presentation. How do you plan to initiate therapy?
Correct Answer: B
Rationale: The correct answer is B: Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support. The patient's presentation is consistent with acute promyelocytic leukemia (APL), indicated by the presence of Auer rod-containing blasts, very bright CD33 expression, and coagulopathy (elevated INR, low fibrinogen, elevated D-dimer). APL is associated with a specific genetic abnormality involving the PML-RARA fusion gene. ATRA is the mainstay of induction therapy for APL as it induces differentiation of leukemic promyelocytes. Aggressive management of coagulopathy is crucial to prevent life-threatening bleeding complications.
Incorrect answers:
A: Performing a lumbar puncture is not necessary at this stage as the patient's symptoms and laboratory findings point towards APL, not central nervous system involvement.
C: Dexamethasone and hydroxyurea are not