Atypical response to all-trans retinoic acid in a der(5)t(5;17) acute promyelocytic leukemia

M. J. Mozziconacci, C. Liberatore, F. Grignani, D. Sainty, P. G. Pelicci, F. Birg, M. Lafage-Pochitaloff

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Typical acute promyelocytic leukemia (APL) is associated with the t(15;17) translocation, expression of a PML/RARA fusion transcript, and responsiveness to all-trans retinoic acid (ATRA). Rare APL cases implicating the RARA but not the PML gene have been reported. Cases with t(11;17)(q23;q21) which fuses the PLZF and RARA genes do not respond to ATRA. In contrast, cases with t(11;17)(q13;q21) and t(5;17)(q35;q21) which fuse RARA with NuMA and NPM, respectively, were reported to be sensitive to ATRA. We described previously an APL case with an unbalanced t(5;17) implicating RARA but neither PML nor PLZF. Here, we show that in this case: (1) the NPM gene is not involved, as demonstrated by RT-PCR and Southern blot; (2) response to ATRA in vitro is atypical, as demonstrated by morphological and functional maturation assays; and (3) PML nuclear bodies are not disrupted, as evidenced by immunofluorescence staining.

Original languageEnglish
Pages (from-to)862-868
Number of pages7
Issue number6
Publication statusPublished - 1999


  • Acute promyelocytic leukemia
  • All-trans retinoic acid
  • Der(5)t(5;17)
  • PML nuclear bodies
  • RARA

ASJC Scopus subject areas

  • Hematology
  • Cancer Research


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