Stem cell transplantation can provide durable disease control in Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
Menée sur 39 patients européens atteints d'une tumeur à cellules plasmacytoïdes dendritiques blastiques, cette étude rétrospective évalue les effets d'une greffe allogène ou autologue de cellules souches sur la survie des patients
Patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN) have a poor prognosis with conventional chemotherapy. We retrospectively analyzed the outcome of patients with BPDCN who underwent allogeneic stem cell transplantation (allo-SCT) or autologous stem cell transplantation (auto-SCT). A total of 39 patients (allo-SCT 34, auto-SCT 5) were identified in the European Group for Blood and Marrow Transplantation registry. The 34 allo-SCT patients had a median age of 41 years (range, 10-70) and were transplanted from sibling (n=11) or unrelated donors (n=23) between 2003 and 2009. Myeloablative conditioning (MAC) was applied in 74%. Nineteen allo-SCT patients (56%) were transplanted in first complete remission. Three-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, whilst age, donor source and chronic graft-versus-host disease had no significant impact. High-dose therapy followed by allo-SCT from related or unrelated donors can provide durable remission even in elderly patients with BPDCN. It remains to be shown, however, if graft-versus-malignancy effects can contribute significantly to BPDCN control after allo-SCT.