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

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.

Figure 1.

(A) Overall survival (OS) of the whole population (43 patients). The median survival was 8.7 months (range, 0.2-32.9). (B) OS in the 17 patients who reached complete remission (CR) after induction compared to patients in partial remission (PR) or with resistant disease. The median OS was 14 months (range, 2-32.9) and 4.5 months (range, 0.2-19.5), respectively (P<0.0001).

Figure 2.

Disease-free survival (DFS) in the 17 patients achieving complete remission. The median DFS was 8.6 months (range, 1-16.5).

Figure 3.

Overall survival (OS) according to types of induction therapy. The median OS was 12.3 months (range 1-32.9) in patients who received an ALL/lymphoma-type regimen and 7.1 months (range, 0.2-19.5) in those treated with an AML-type regimen (P=0.02).