Purpose. 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 leukemic presentation at onset of disease. Design and Methods. A retrospective multicenter study was performed from 2005-2011 in 28 Italian hematology divisions. Results. Forty-three cases were collected. Forty-one patients received an induction therapy, consisting of an acute myeloid leukemia-type regimen in 26 pts (60%) and acute lymphoid leukemia/lymphoma-type in 15 patients (35%). Six patients (14%) underwent allogeneic hemopoietic stem cell transplant. A complete remission was achieved in 17 patients (41%). There were 7 complete remission after the acute myeloid leukemia-type treatment and 10 complete remission after the acute lymphoid leukemia/lymphoma-type regimen, with a significant advantage for acute lymphoid leukemia/lymphoma-type chemotherapy (p=0.02). Relapse occurred in 6 of the 17 patients (35%) achieved complete remission, more frequently after acute lymphoid leukemia/lymphoma-type chemotherapy. The median overall survival was 8.7 months (0.2-32.9). The patients treated with acute myeloid leukemia-type regimen had an overall survival of 7.1 months (0.2-19.5), and the patients receiving acute lymphoid leukemia/lymphoma-type regimen had an overall survival of 12.3 months (1-32.9) (p=0.02). In allogeneic hemopoietic stem cell transplant patients, the median overall survival was 22.7 months (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). Conclusions: Blastic Plasmacytoid Dendritic Cell Neoplasm with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia. The rarity of this leukemic form does not allow prospective clinical trials able to identify the better therapeutic strategy, that at present is based on the clinicians experience
Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study / Pagano L;Valentini CG;Pulsoni A;Fisogni S;Carluccio P;Mannelli F;Lunghi M;Pica G;Onida F;Cattaneo C;Piccaluga P;Di Bona E;Todisco E;Musto P;Spadea A;D'Arco A;Pileri S;Leone G;Amadori S;Facchetti F. - In: HAEMATOLOGICA. - ISSN 0390-6078. - ELETTRONICO. - (2012), pp. 1-2. [10.3324/haematol.2012.072645]
Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study.
MANNELLI, FRANCESCO;
2012
Abstract
Purpose. 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 leukemic presentation at onset of disease. Design and Methods. A retrospective multicenter study was performed from 2005-2011 in 28 Italian hematology divisions. Results. Forty-three cases were collected. Forty-one patients received an induction therapy, consisting of an acute myeloid leukemia-type regimen in 26 pts (60%) and acute lymphoid leukemia/lymphoma-type in 15 patients (35%). Six patients (14%) underwent allogeneic hemopoietic stem cell transplant. A complete remission was achieved in 17 patients (41%). There were 7 complete remission after the acute myeloid leukemia-type treatment and 10 complete remission after the acute lymphoid leukemia/lymphoma-type regimen, with a significant advantage for acute lymphoid leukemia/lymphoma-type chemotherapy (p=0.02). Relapse occurred in 6 of the 17 patients (35%) achieved complete remission, more frequently after acute lymphoid leukemia/lymphoma-type chemotherapy. The median overall survival was 8.7 months (0.2-32.9). The patients treated with acute myeloid leukemia-type regimen had an overall survival of 7.1 months (0.2-19.5), and the patients receiving acute lymphoid leukemia/lymphoma-type regimen had an overall survival of 12.3 months (1-32.9) (p=0.02). In allogeneic hemopoietic stem cell transplant patients, the median overall survival was 22.7 months (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). Conclusions: Blastic Plasmacytoid Dendritic Cell Neoplasm with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia. The rarity of this leukemic form does not allow prospective clinical trials able to identify the better therapeutic strategy, that at present is based on the clinicians experience
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