A Pathogenic NRAS c.38G>A (p.G13D) Mutation in RARA Translocation-negative Acute Promyelocytic-like Leukemia with Concomitant Myelodysplastic Syndrome

Intern Med. 2023 May 1;62(9):1329-1334. doi: 10.2169/internalmedicine.0174-22. Epub 2022 Sep 21.

Abstract

An acute promyelocytic leukemia (APL) patient not demonstrating the retinoic acid receptor α (RARA) translocation is rare. A 76-year-old man was diagnosed with myelodysplastic syndrome (MDS). After a year, abnormal promyelocytes were detected with pancytopenia and disseminated intravascular coagulopathy. Morphologically, the patient was diagnosed with APL; however, a genetic examination failed to detect RARA translocation. Thereafter, whole-genome sequencing revealed an NRAS missense mutation [c.38G>A (p.G13D)]. This mutation was not detected in posttreatment bone marrow aspirate, despite residual MDS. Few reports are available on similar cases. Furthermore, the NRAS c.38G>A mutation may be a novel pathogenic variant exacerbating RARA translocation-negative acute promyelocytic-like leukemia.

Keywords: NRAS; acute promyelocytic leukemia (APL); acute promyelocytic-like leukemia (APL-like); myelodysplastic syndrome (MDS); retinoic acid receptor α (RARA) translocation-negative APL.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • GTP Phosphohydrolases / genetics
  • Granulocyte Precursor Cells / pathology
  • Humans
  • Leukemia, Promyelocytic, Acute* / complications
  • Leukemia, Promyelocytic, Acute* / diagnosis
  • Leukemia, Promyelocytic, Acute* / genetics
  • Male
  • Membrane Proteins / genetics
  • Mutation / genetics
  • Myelodysplastic Syndromes* / complications
  • Myelodysplastic Syndromes* / genetics
  • Translocation, Genetic

Substances

  • GTP Phosphohydrolases
  • Membrane Proteins
  • NRAS protein, human
  • RARA protein, human