Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Livio Pagano(Catholic University of America), Caterina Giovanna Valentini(Catholic University of America), Alessandro Pulsoni, Simona Fisogni(Brescia University), P. Carluccio(University of Bari Aldo Moro), Francesco Mannelli(University of Florence), Monia Lunghi(Università degli Studi del Piemonte Orientale “Amedeo Avogadro”), Gian-Matteo Pica(Ospedale Policlinico San Martino), Francesco Onida(Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico), Chiara Cattaneo(Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia), Pier Paolo Piccaluga(University of Bologna), E. Di Bona(Ospedale San Bortolo), Elisabetta Todisco(Humanitas University), Pellegrino Musto(Istituti di Ricovero e Cura a Carattere Scientifico), Antonio Spadea(National Cancer Institute), Alfonso Maria D’Arco(National Cancer Institute), S Pileri(University of Bologna), Giuseppe Leone(Catholic University of America), Sergio Amadori(University of Rome Tor Vergata), Fabio Facchetti(Brescia University), for GIMEMA-ALWP (Gruppo Italiano Malattie EMatologiche dell'Adulto, Acute Leukemia Working Party)
Haematologica
October 12, 2012
10.3324/haematol.2012.072645
Cited by 335Open Access
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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.

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