Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: An italian multicenter study

Livio Pagano, Caterina Giovanna Valentini, Alessandro Pulsoni, Simona Fisogni, Paola Carluccio, Francesco Mannelli, Monia Lunghi, Gianmatteo Pica, Francesco Onida, Chiara Cattaneo, Pier Paolo Piccaluga, Eros Di Bona, Elisabetta Todisco, Pellegrino Musto, Antonio Spadea, Alfonso D'Arco, Stefano Pileri, Giuseppe Leone, Sergio Amadori, Fabio Facchetti

Research output: Contribution to journalArticle

Abstract

The objective of this study was to evaluate the clinical features, prognostic factors, and efficacy of treatments in patients with blastic plasmacytoid dendritic cell neoplasm with a leukemic presentation at onset of the disease. In order to do this, a retrospective multicenter study was performed from 2005-2011 in 28 Italian hematology divisions in which 43 cases were collected. Forty-one patients received an induction therapy, consisting of an acute myeloid leukemia-type regimen in 26 patients (60%) and acute lymphoid leukemia/lymphoma-type regimen in 15 patients (35%). Six patients (14%) underwent allogeneic hematopoietic stem cell transplantation. Seventeen patients (41%) achieved a complete remission: seven after acute myeloid leukemia-type treatment and 10 after an acute lymphoid leukemia/lymphoma-type regimen, with a significant advantage for acute lymphoid leukemia/lymphoma-type chemotherapy (P=0.02). Relapse occurred in six of the 17 patients (35%) who achieved complete remission, more frequently after acute lymphoid leukemia/lymphoma-type chemotherapy. The median overall survival was 8.7 months (range, 0.2-32.9). The patients treated with an acute myeloid leukemia-type regimen had an overall survival of 7.1 months (range, 0.2-19.5), whereas that of the patients receiving acute lymphoid leukemia/lymphoma-type chemotherapy was 12.3 months (range, 1-32.9) (P=0.02). The median overall survival of the allogeneic hematopoietic stem cell transplant recipients was 22.7 months (range, 12-32.9), and these patients had a significant survival advantage compared to the non-transplanted patients (median 7.1 months, 0.2-21.3; P=0.03). In conclusion, blastic plasmacytoid dendritic cell neoplasm with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia. The rarity of this disease does not enable prospective clinical trials to identify the better therapeutic strategy, which, at present, is based on clinicians' experience.

Original languageEnglish
Pages (from-to)239-246
Number of pages8
JournalHaematologica
Volume98
Issue number2
DOIs
Publication statusPublished - Feb 1 2013

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Dendritic Cells
Multicenter Studies
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Neoplasms
Lymphoma
Acute Myeloid Leukemia
Drug Therapy
Bone Marrow Neoplasms
Survival
Hematopoietic Stem Cell Transplantation
Hematology
Hematopoietic Stem Cells
Leukemia
Therapeutics
Retrospective Studies
Clinical Trials
Transplants
Recurrence

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

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Pagano, L., Valentini, C. G., Pulsoni, A., Fisogni, S., Carluccio, P., Mannelli, F., ... Facchetti, F. (2013). Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: An italian multicenter study. Haematologica, 98(2), 239-246. https://doi.org/10.3324/haematol.2012.072645

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation : An italian multicenter study. / Pagano, Livio; Valentini, Caterina Giovanna; Pulsoni, Alessandro; Fisogni, Simona; Carluccio, Paola; Mannelli, Francesco; Lunghi, Monia; Pica, Gianmatteo; Onida, Francesco; Cattaneo, Chiara; Piccaluga, Pier Paolo; Di Bona, Eros; Todisco, Elisabetta; Musto, Pellegrino; Spadea, Antonio; D'Arco, Alfonso; Pileri, Stefano; Leone, Giuseppe; Amadori, Sergio; Facchetti, Fabio.

In: Haematologica, Vol. 98, No. 2, 01.02.2013, p. 239-246.

Research output: Contribution to journalArticle

Pagano, L, Valentini, CG, Pulsoni, A, Fisogni, S, Carluccio, P, Mannelli, F, Lunghi, M, Pica, G, Onida, F, Cattaneo, C, Piccaluga, PP, Di Bona, E, Todisco, E, Musto, P, Spadea, A, D'Arco, A, Pileri, S, Leone, G, Amadori, S & Facchetti, F 2013, 'Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: An italian multicenter study', Haematologica, vol. 98, no. 2, pp. 239-246. https://doi.org/10.3324/haematol.2012.072645
Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: An italian multicenter study. Haematologica. 2013 Feb 1;98(2):239-246. https://doi.org/10.3324/haematol.2012.072645
Pagano, Livio ; Valentini, Caterina Giovanna ; Pulsoni, Alessandro ; Fisogni, Simona ; Carluccio, Paola ; Mannelli, Francesco ; Lunghi, Monia ; Pica, Gianmatteo ; Onida, Francesco ; Cattaneo, Chiara ; Piccaluga, Pier Paolo ; Di Bona, Eros ; Todisco, Elisabetta ; Musto, Pellegrino ; Spadea, Antonio ; D'Arco, Alfonso ; Pileri, Stefano ; Leone, Giuseppe ; Amadori, Sergio ; Facchetti, Fabio. / Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation : An italian multicenter study. In: Haematologica. 2013 ; Vol. 98, No. 2. pp. 239-246.
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AU - Lunghi, Monia

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AU - Onida, Francesco

AU - Cattaneo, Chiara

AU - Piccaluga, Pier Paolo

AU - Di Bona, Eros

AU - Todisco, Elisabetta

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AU - Spadea, Antonio

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