Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission.
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.
Male
H J
HJ
Blood and Marrow Transplantation Program, University of Kansas Medical Center, Kansas City, KS 66205, USA.
Davis
M B
MB
McGuirk
J P
JP
Abhyankar
S
S
Aljitawi
O S
OS
Zhang
D
D
Ganguly
Siddhartha
S
D000970:Antineoplastic Agents / Q000627:therapeutic use; D001853:Bone Marrow; D003713:Dendritic Cells / Q000473:pathology*; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007938:Leukemia / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012878:Skin Neoplasms / Q000628:therapy; D018183:Transplantation Chimera; D014184:Transplantation, Homologous
D000970:Antineoplastic Agents