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[Blastic plasmacytoid dendritic cell neoplasm: two cases report and review of literatures]
Gang An1, Jun-Yuan Qi, De-Hui Zou
1Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin 300020, China.
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.
Conclusion:
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.
