Haematologica (Feb 2013)

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

DOI
https://doi.org/10.3324/haematol.2012.072645
Journal volume & issue
Vol. 98, no. 2

Abstract

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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.