Identification of DNA rearrangements at the retinoic acid receptor-alpha (RAR-alpha) locus in all patients with acute promyelocytic leukemia (APL) and mapping of APL breakpoints within the RAR-alpha second intron. Italian Cooperative Study Group "GIMEMA"

Blood. 1992 Jun 15;79(12):3331-6.

Abstract

Seventy patients with acute promyelocytic leukemia (APL) were characterized at the DNA level using genomic retinoic acid receptor-alpha (RAR-alpha) probes on Southern blot experiments. Sixty-two cases were defined as M3 according to the French-American-British (FAB) criteria, and eight had a diagnosis of microgranular or variant (M3v) APL. The use of two restriction enzymes and three probes exploring the second intron of the RAR-alpha gene allowed us to detect specific abnormal DNA fragments in every case, with clustering of rearrangements within the 20-kb intronic region between RAR-alpha exons II and III. A more detailed mapping of APL breakpoints was performed in 52 cases in which three EcoRI subregions of the RAR-alpha second intron were analyzed with corresponding probes. Comparison of clinical and hematological features in the three subgroups of patients with distinct RAR-alpha breakpoints did not show significant differences regarding age, peripheral blood (PB) counts, presence of coagulopathy, or FAB classification (M3 v M3v). Interestingly, a significant difference was observed in the M/F ratio of the three subgroups, with a higher incidence of rearrangements at the 5' end of the RAR-alpha second intron in female patients, and more frequent 3' breakpoints in males. The results of this study indicate that a unique genomic alteration consistently occurs on the 17q- derivative of the APL specific t(15;17) aberration. Moreover, the clinical relevance of RAR-alpha gene analysis both at diagnosis and in follow-up studies is further emphasized.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Blotting, Southern
  • Carrier Proteins / genetics*
  • Child
  • Child, Preschool
  • Chromosome Mapping
  • DNA / genetics*
  • Deoxyribonuclease EcoRI
  • Deoxyribonuclease HindIII
  • Exons
  • Female
  • Gene Rearrangement*
  • Humans
  • Infant
  • Introns
  • Leukemia, Promyelocytic, Acute / genetics*
  • Male
  • Middle Aged
  • Receptors, Retinoic Acid

Substances

  • Carrier Proteins
  • Receptors, Retinoic Acid
  • DNA
  • Deoxyribonuclease EcoRI
  • Deoxyribonuclease HindIII