PML-RARA fusion resulting from a cryptic insertion of RARA gene into PML gene without the reciprocal RARA-PML fusion: clinical, cytogenetic, and molecular characterization and prognosis

Eur J Haematol. 2014 Oct;93(4):354-8. doi: 10.1111/ejh.12326. Epub 2014 Apr 26.

Abstract

We describe a case of acute promyelocytic leukemia in a 61-yr-old woman with a cryptic insertion of RARA gene into PML gene. Using a combination of cytogenetic and molecular methods, we confirmed the insertion and presence of the PML-RARA transcript and lack of the reciprocal RARA-PML transcript. Although such cryptic insertions leading to a PML-RARA fusion have been previously reported, we show that such variant insertions, based on our case, appear to have the same prognostic significance as the classical t(15;17)(q22;q21).

Keywords: acute promyelocytic leukemia; cryptic PML-RARA; fluorescence in situ hybridization; karyotype; reverse transcriptase-polymerase chain reaction.

Publication types

  • Case Reports

MeSH terms

  • Abnormal Karyotype
  • Bone Marrow / pathology
  • Cytogenetic Analysis
  • Female
  • Humans
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Leukemia, Promyelocytic, Acute / diagnosis*
  • Leukemia, Promyelocytic, Acute / genetics*
  • Middle Aged
  • Mutagenesis, Insertional*
  • Oncogene Proteins, Fusion / genetics*
  • Prognosis
  • Translocation, Genetic

Substances

  • Oncogene Proteins, Fusion
  • promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein