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June 1999, Volume 13, Number 6, Pages 862-868
Table of contents    Previous  Abstract  Next   Article  PDF
Original manuscript
Atypical response to all-trans retinoic acid in a der(5)t(5;17) acute promyelocytic leukemia
M-J Mozziconacci1,2, C Liberatore3, F Grignani3, D Sainty1, P G Pelicci3,4, F Birg2 and M Lafage-Pochitaloff1,2

1Department of Biology and Hematology, Institute Paoli-Calmettes, Marseille, France

2INSERM U119, Marseille, France

3Istituto di Clinica Medica, Policlinico Monteluce, University of Perugia, Italy

4European Institute of Oncology, Milan, Italy

Correspondence to: M Lafage-Pochitaloff, Institut Paoli- Calmettes and INSERM U119, 232 Bd de Sainte-Marguerite, 13273 Marseille Cedex 9 France; Fax: 33 (0) 4 91 22 35 44

Abstract

Typical acute promyelocytic leukemia (APL) is associated with the t(15;17) translocation, expression of a PML/RARA fusion transcript, and responsiveness to all-transretinoic acid (ATRA). Rare APL cases implicating the RARA but not the PMLgene 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.

Keywords

acute promyelocytic leukemia; RARA; der(5)t(5; 17); all-trans retinoic acid; PML nuclear bodies

Received 8 October 1998; accepted 10 February 1999
June 1999, Volume 13, Number 6, Pages 862-868
Table of contents    Previous  Abstract  Next   Article  PDF