title: Sustained remission of blastic plasmacytoid dendritic cell neoplasm after unrelated allogeneic stem cell transplantation--a single center experience.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic neoplasm which typically presents with skin infiltrates with or without lymphadenopathy and bone marrow involvement. No standard of care exists for this aggressive disease and prognosis is particularly poor. Here, we present our experience with nine BPDCN patients diagnosed at our institution between 2005 and 2012. BPDCN patients were identified in the databases at the Department of Hematology and Oncology, the Department of Dermatology, and the Institute of Pathology at the Otto-von-Guericke-University Magdeburg. There were six male and three female patients with a median age at diagnosis of 66 years. Sites involved were skin (five cases), lymph nodes (five cases), and bone marrow (five cases). Treatments varied from single agent chemotherapy to polychemotherapy and allogeneic stem cell transplantation for consolidation. The three patients that were treated with acute leukemia-type induction therapy followed by allogeneic stem cell transplantation (one after standard conditioning and two after reduced intensity conditioning using fludarabine in combination with thiotepa) achieved sustained remissions and are alive with a follow-up of 8, 35, and 41 months. In contrast, median survival in the less intensively treated patients was only 9.5 (range 1 to 29) months.
authors.affiliation: Department of Hematology and Oncology, Medical Center, Otto-von-Guericke-University Magdeburg, Leipziger Str. 44, 39120, Magdeburg, Germany, thomas.heinicke@med.ovgu.de.
authors.forename: Thomas
authors.initials: T
authors.lastname: Heinicke
authors.forename: Heiko
authors.initials: H
authors.lastname: Hütten
authors.forename: Thomas
authors.initials: T
authors.lastname: Kalinski
authors.forename: Ingolf
authors.initials: I
authors.lastname: Franke
authors.forename: Bernd
authors.initials: B
authors.lastname: Bonnekoh
authors.forename: Thomas
authors.initials: T
authors.lastname: Fischer
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059248:Chemoradiotherapy / Q000379:methods; D003131:Combined Modality Therapy; D003713:Dendritic Cells / Q000473:pathology*; D005260:Female; D019337:Hematologic Neoplasms / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000628:therapy*; D016019:Survival Analysis; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D061349:Unrelated Donors; D055815:Young Adult