title: Unrelated SCT induces long-term remission in patients with blastic plasmacytoid dendritic cell neoplasm.
abstract: Blastic plasmacytoid DC neoplasm (BPDCN) is a rare haematopoietic malignancy with an aggressive behaviour. We evaluated five patients allografted as consolidative treatment with an unrelated donor in first or subsequent remission. Four patients received a reduced intensity-conditioning regimen because of age or co-morbidities. As the stem cell sources, two umbilical cord blood-(UCB), two PBSC- and one BM graft were used. No GVHD was observed in the patients who received a UCB graft. However, both developed a post-transplant-associated lymphoproliferative disease. So far, only one patient has experienced relapse and was consecutively treated by escalated donor lymphocyte infusions (DLI). A potent graft-versus-leukaemia (GVL) effect was induced leading to a 17-month-long CR. Four patients are still in ongoing CR with median disease-free and overall survivals of 17 and 21 months. Thus, allogeneic SCT in BPDCN offers a potential curative option for patients with a compatible donor. UCB is an attractive alternative as a stem cell source. For relapsing patients, DLI can exert a powerful GVL effect.
authors.affiliation: Department of Internal Medicine, Division of Haematology, Medical University of Graz, Graz, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Unteregger
authors.forename: A
authors.initials: A
authors.lastname: Valentin
authors.forename: W
authors.initials: W
authors.lastname: Zinke-Cerwenka
authors.forename: K
authors.initials: K
authors.lastname: Troppan
authors.forename: A
authors.initials: A
authors.lastname: Deutsch
authors.forename: L
authors.initials: L
authors.lastname: Cerroni
authors.forename: W
authors.initials: W
authors.lastname: Linkesch
authors.forename: P
authors.initials: P
authors.lastname: Neumeister
mesh_terms: D000328:Adult; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D020544:Graft vs Leukemia Effect; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D054219:Neoplasms, Plasma Cell / Q000401:mortality / Q000628:therapy*; D033581:Stem Cell Transplantation; D015996:Survival Rate; D013997:Time Factors; D019172:Transplantation Conditioning; D061349:Unrelated Donors