[Blastic plasmacytoid dendritic cell neoplasm: two cases report and review of literatures].
OBJECTIVE
To identify the clinical and pathological features of blastic plasmacytoid dendritic cell neoplasm (BPDC).


METHODS
The characteristics of BPDC hematodermic neoplasm were discussed with a report of two new cases and review the literatures.


RESULTS
Both patients presented with skin nodules and the tumors were CD(4)(+) and CD(56)(+). Lineage specific markers for B- and T-cell were negative and the tumors did not express myeloperoxidase. Systemic chemotherapy resulted in complete remission, but the disease relapsed quickly and were unresponsive to further chemotherapy. The patients died 26 months and 11 months respectively after diagnosis.


CONCLUSIONS
BPDC hematodermic neoplasm is a rare subtype of lymphoma with distinct clinicopathologic and immunophenotypic features. The disease often has a fulminant course with a poor prognosis. More recent studies suggest that there is a derivation from a plasmacytoid dendritic cell precursor.
An
Gang
G
Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin 300020, China.
Qi
Jun-Yuan
JY
Zou
De-Hui
DH
Zhao
Yao-Zhong
YZ
Chen
Hui-Shu
HS
Qiu
Lu-Gui
LG
D003713:Dendritic Cells / Q000473:pathology*; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000473:pathology*