Unrelated SCT induces long-term remission in patients with blastic plasmacytoid dendritic cell neoplasm

Bone Marrow Transplant. 2013 Jun;48(6):799-802. doi: 10.1038/bmt.2012.242. Epub 2012 Dec 3.

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.

MeSH terms

  • Adult
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Graft vs Leukemia Effect*
  • Humans
  • Lymphoproliferative Disorders / etiology
  • Lymphoproliferative Disorders / mortality
  • Lymphoproliferative Disorders / therapy*
  • Male
  • Middle Aged
  • Neoplasms, Plasma Cell / mortality
  • Neoplasms, Plasma Cell / therapy*
  • Stem Cell Transplantation*
  • Survival Rate
  • Time Factors
  • Transplantation Conditioning*
  • Unrelated Donors