Complete remission through blast cell differentiation in PLZF/RARα-positive acute promyelocytic leukemia: In vitro and in vivo studies

Maria C. Petti, Francesco Fazi, Massimo Gentile, Daniela Diverio, Paolo De Fabritiis, M. Stefania De Propris, Roberto Fiorini, Maria A. Aloe Spiriti, Fabrizio Padula, Pier Giuseppe Pelicci, Clara Nervi, Francesco Lo Coco

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Abstract

Acute leukemia with the t(11;17) expressing the PLZF-RARα gene fusion is a rare variant of acute promyelocytic leukemia (APL) that has been associated with poor clinical response to all-trans retinoic acid (ATRA) treatment. However, some recent reports have put into question the absolute refractoriness of this leukemia to ATRA. We describe here a patient with PLZF/RARα APL who was treated at relapse with ATRA and low-dose hydroxyurea. Complete hematologic remission was obtained through differentation of leukemic blasts, as proven by morphologic, immunophenophenotypic, and genetic studies carried out in sequential bone marrow samples. Moreover, in vitro studies indicated that blast differentiation was potentiated by the addition of the histone deacetylase inhibitor tricostatin A, but not of hydroxyurea, to ATRA. Our findings indicate that the maturation block may be overcome and terminal differentiation obtained in this leukemia subset and support the view that sensitivity/refractoriness of this form to ATRA should be revisited.

Original languageEnglish
Pages (from-to)1065-1067
Number of pages3
JournalBlood
Volume100
Issue number3
DOIs
Publication statusPublished - Aug 1 2002

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Acute Promyelocytic Leukemia
Tretinoin
Cell Differentiation
Leukemia
Hydroxyurea
trichostatin A
Histone Deacetylase Inhibitors
Gene Fusion
Bone
Fusion reactions
Genes
Bone Marrow
In Vitro Techniques
Recurrence

ASJC Scopus subject areas

  • Hematology

Cite this

Complete remission through blast cell differentiation in PLZF/RARα-positive acute promyelocytic leukemia : In vitro and in vivo studies. / Petti, Maria C.; Fazi, Francesco; Gentile, Massimo; Diverio, Daniela; De Fabritiis, Paolo; De Propris, M. Stefania; Fiorini, Roberto; Aloe Spiriti, Maria A.; Padula, Fabrizio; Pelicci, Pier Giuseppe; Nervi, Clara; Lo Coco, Francesco.

In: Blood, Vol. 100, No. 3, 01.08.2002, p. 1065-1067.

Research output: Contribution to journalArticle

Petti, MC, Fazi, F, Gentile, M, Diverio, D, De Fabritiis, P, De Propris, MS, Fiorini, R, Aloe Spiriti, MA, Padula, F, Pelicci, PG, Nervi, C & Lo Coco, F 2002, 'Complete remission through blast cell differentiation in PLZF/RARα-positive acute promyelocytic leukemia: In vitro and in vivo studies', Blood, vol. 100, no. 3, pp. 1065-1067. https://doi.org/10.1182/blood-2001-12-0368
Petti, Maria C. ; Fazi, Francesco ; Gentile, Massimo ; Diverio, Daniela ; De Fabritiis, Paolo ; De Propris, M. Stefania ; Fiorini, Roberto ; Aloe Spiriti, Maria A. ; Padula, Fabrizio ; Pelicci, Pier Giuseppe ; Nervi, Clara ; Lo Coco, Francesco. / Complete remission through blast cell differentiation in PLZF/RARα-positive acute promyelocytic leukemia : In vitro and in vivo studies. In: Blood. 2002 ; Vol. 100, No. 3. pp. 1065-1067.
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