ATI Hematologic System Test Related

Review ATI Hematologic System Test related questions and content

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?

  • A. Perform a lumbar puncture to determine leukemic involvement, then proceed with induction chemotherapy.
  • B. Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support.
  • C. Start dexamethasone and hydroxyurea immediately while aggressively managing coagulopathy with blood product support.
  • D. Start induction chemotherapy, obtain HLA typing, and start a donor search because of the poor prognosis associated with this leukemic phenotype.
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.

Rationale:
1. The clinical presentation describes acute promyelocytic leukemia (APL), characterized by Auer rod-containing blasts, coagulopathy (elevated INR, D-dimer), and CD33 positivity. ATRA is the standard treatment for APL.
2. ATRA induces differentiation of leukemic promyelocytes, reducing the risk of disseminated intravascular coagulation (DIC) and bleeding.
3. Aggressively managing the coagulopathy with blood product support is crucial to prevent hemorrhagic complications.

Summary:
A: Not needed as the clinical presentation already suggests APL. Lumbar puncture is not indicated for APL diagnosis.
C: Dexamethasone and hydroxyurea are not standard treatments for APL. Immediate ATRA therapy is preferred.
D