Use of whole-genome sequencing to diagnose a cryptic fusion oncogene

JAMA. 2011 Apr 20;305(15):1577-84. doi: 10.1001/jama.2011.497.

Abstract

Context: Whole-genome sequencing is becoming increasingly available for research purposes, but it has not yet been routinely used for clinical diagnosis.

Objective: To determine whether whole-genome sequencing can identify cryptic, actionable mutations in a clinically relevant time frame.

Design, setting, and patient: We were referred a difficult diagnostic case of acute promyelocytic leukemia with no pathogenic X-RARA fusion identified by routine metaphase cytogenetics or interphase fluorescence in situ hybridization (FISH). The case patient was enrolled in an institutional review board-approved protocol, with consent specifically tailored to the implications of whole-genome sequencing. The protocol uses a "movable firewall" that maintains patient anonymity within the entire research team but allows the research team to communicate medically relevant information to the treating physician.

Main outcome measures: Clinical relevance of whole-genome sequencing and time to communicate validated results to the treating physician.

Results: Massively parallel paired-end sequencing allowed identification of a cytogenetically cryptic event: a 77-kilobase segment from chromosome 15 was inserted en bloc into the second intron of the RARA gene on chromosome 17, resulting in a classic bcr3 PML-RARA fusion gene. Reverse transcription polymerase chain reaction sequencing subsequently validated the expression of the fusion transcript. Novel FISH probes identified 2 additional cases of t(15;17)-negative acute promyelocytic leukemia that had cytogenetically invisible insertions. Whole-genome sequencing and validation were completed in 7 weeks and changed the treatment plan for the patient.

Conclusion: Whole-genome sequencing can identify cytogenetically invisible oncogenes in a clinically relevant time frame.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Chromosome Breakpoints
  • Chromosomes, Human, Pair 15 / genetics
  • Chromosomes, Human, Pair 17 / genetics
  • Gene Fusion
  • Genome, Human
  • Humans
  • Introns
  • Leukemia, Promyelocytic, Acute / genetics*
  • Leukemia, Promyelocytic, Acute / therapy
  • Male
  • Nuclear Proteins / genetics*
  • Oncogene Proteins, Fusion / genetics*
  • Promyelocytic Leukemia Protein
  • Receptors, Retinoic Acid / genetics*
  • Retinoic Acid Receptor alpha
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA*
  • Transcription Factors / genetics*
  • Tumor Suppressor Proteins / genetics*

Substances

  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • Promyelocytic Leukemia Protein
  • RARA protein, human
  • Receptors, Retinoic Acid
  • Retinoic Acid Receptor alpha
  • Transcription Factors
  • Tumor Suppressor Proteins
  • PML protein, human