Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission

Int J Hematol. 2010 Sep;92(2):398-400. doi: 10.1007/s12185-010-0655-0. Epub 2010 Aug 11.

Abstract

Blastic plasmacytoid dendritic cell (BPDC) neoplasm is a rare but clinically aggressive tumor known to be derived from the precursors of plasmacytoid dendritic cells (CD123+) with a high frequency of cutaneous and bone marrow involvement. Though majority of the patients initially respond to multi-agent chemotherapy, most would relapse within a year. We hereby report a patient with disseminated cutaneous BPDC with marrow involvement diagnosed by typical histo-pathological and flow-cytometric findings. He was subsequently treated with leukemia type induction regimen followed by allogeneic stem cell transplantation in first complete remission. He is now 18 months posttransplantation with continued remission with full donor chimerism. We recognize that BPDC with marrow involvement behaves like acute myeloid leukemia and aggressive treatment followed by stem cell transplantation may lead to long-term remission in selected cases.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Bone Marrow
  • Dendritic Cells / pathology*
  • Hematopoietic Stem Cell Transplantation / methods*
  • Humans
  • Leukemia / therapy*
  • Male
  • Middle Aged
  • Remission Induction
  • Skin Neoplasms / therapy
  • Transplantation Chimera
  • Transplantation, Homologous

Substances

  • Antineoplastic Agents