Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation

Abstract

Key Points• BPDCN is a rare hematopoietic malignancy characterized by a poor prognosis and unusual resistance to conventional chemotherapy.• This study indicates that highdose therapy followed by allo-SCT can provide durable disease control in this otherwise inevitably fatal condition. Nineteen allo-SCT patients (56%) received transplantations in first complete remission. The 3-year cumulative incidence of relapse, disease-free survival, and overall survival was 32%, 33%, and 41%, respectively. By univariate comparison, being in first remission at allo-SCT favorably influenced survival, whereas age, donor source, and chronic GVHD had no significant impact. We conclude that high-dose therapy followed by allo-SCT from related or unrelated donors can provide durable remission even in elderly patients with BPDCN. However, it remains to be shown if graft-versus-malignancy effects can contribute significantly to BPDCN control after allo-SCT. (Blood. 2013;121(3): 440-446)

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10‐Color flow cytometry diagnosis and HyperCVAD therapy

“…Recent data suggest that the median OS of patients who received an allogeneic-Hematopoeitic Cell Transplant (allo-HCT) were superior to those who received chemotherapy alone (22.7 months vs. 7.1 months; P 5 0.03) [7]. Allo-HCT when performed, mostly in patients in first CR, yielded 3-and 4-year OS of 41 and 53%, respectively [11,12]. Similar survival benefits may also be obtained with an autologous HCT in first CR [12].…”

In vivo and in vitro sensitivity of blastic plasmacytoid dendritic cell neoplasm to SL-401, an interleukin-3 receptor targeted biologic agent

“…22 IL-3 has also been shown to be a critical survival factor for plasmacytoid dendritic cells. 33 Thus, interference of this pathway by SL-401 may explain the high sensitivity of BPDCN compared with the sensitivity of other myeloid and lymphoid leukemic cells.There is still no consensus on the best therapeutic approach for BPDCN, 3,[12][13][14][15][34][35][36][37][38] and, overall, BPDCN remains a chemotherapy-resistant disease and may also resist the graft-versus-leukemia effect, since 32% of patients relapse after allogeneic hematopoietic cell transplantation according to a recent study. 13 Thus, targeted or immune-based therapies are alternative strategies to treat this aggressive leukemia.…”

Blastic Plasmacytoid Dendritic Cell Neoplasm: Skin and Bone Marrow Infiltration of Three Cases and the Review of the Literature

“…However, in another study of 33 patients, bone marrow infiltration did not affect the prognosis [17]. Weil et al reported that allogeneic bone marrow transplantation in first remission was related to 3-years longer progression-free survival and overall survival (33 and 41 %respectively) [5]. A patient who underwent reduced intensity transplantation had a 4-year survival [16].…”

“…The median survival of patients was 15.2 months in a large case series [4]. Allogeneic or autologous bone marrow transplantation has been recommended by some reports because of the disease's poor prognosis [3,5]. BPDCN usually presents with cutaneous and non-cutaneous involvement.…”