Alkaloid-based regimen is beneficial for acute myeloid leukemia resembling acute promyelocytic leukemia with NUP98/RARG fusion and RUNX1 mutation: A case report

Medicine (Baltimore). 2020 Oct 2;99(40):e22488. doi: 10.1097/MD.0000000000022488.

Abstract

Rationale: Some acute myeloid leukemia (AML) patients present with features mimicking the classical hypergranular subtype of acute promyelocytic leukemia (APL) but without the typical promyelocytic leukemia/retinoic acid receptor α (PML/RARα) rearrangement. Herein, we report an AML patient resembling APL but with nucleoporin 98/retinoid acid receptor gamma gene (NUP98/RARG) fusion transcript and Runt-related transcription factor 1 (RUNX1) mutation.

Patient concerns: An 18-year-old male presented at the hospital with a diagnosis of AML.

Diagnoses: The patient was diagnosed with bone marrow examination. Bone marrow smear displayed 90.5% promyelocytes. Fluorescence in situ hybridization analysis failed to detect the PML/RARα fusion transcript or RARα amplification. While real-time polymerase chain reaction showed positivity for the NUP98/RARG fusion transcript. G-banding karyotype analysis showed a normal karyotype.

Interventions: The patient showed resistance to arsenic trioxide and standard 3 + 7 chemotherapy, but eventually achieved complete remission through the Homoharringtonine, Cytarabine, and Aclarubicin chemotherapy.

Outcomes: These measures resulted in a rapid response and disease control.

Lessons: Acute myeloid leukemia with the NUP98/RARG fusion gene and the RUNX1 mutation may be a special subtype of AML and may benefit from the alkaloid-based regimen.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Core Binding Factor Alpha 2 Subunit / genetics
  • Diagnosis, Differential
  • Humans
  • Leukemia, Myeloid, Acute / diagnosis
  • Leukemia, Myeloid, Acute / drug therapy*
  • Leukemia, Myeloid, Acute / genetics*
  • Leukemia, Promyelocytic, Acute / diagnosis
  • Male
  • Nuclear Pore Complex Proteins / genetics
  • Receptors, Retinoic Acid / genetics
  • Retinoic Acid Receptor gamma

Substances

  • Core Binding Factor Alpha 2 Subunit
  • Nuclear Pore Complex Proteins
  • Nup98 protein, human
  • RUNX1 protein, human
  • Receptors, Retinoic Acid