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Published on: September 25, 2018
Blastic plasmacytoid dendritic cell neoplasm: a single-center experience
Ho Jung An1, Dok Hyun Yoon, Shin Kim
1Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 138-736, South Korea.
Annals of Hematology
|November 9, 2012
Summary
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive cancer. Skin involvement in BPDCN patients was associated with improved survival, warranting further prognostic investigation.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy.
- Recently recognized as a distinct entity, BPDCN requires further characterization of its clinical and pathological features.
Purpose of the Study:
- To review clinical, pathological features, and treatment outcomes of BPDCN.
- To investigate the prognostic significance of skin involvement in BPDCN patients.
Main Methods:
- Retrospective analysis of seven BPDCN patients treated between 2000 and 2010.
- Review of clinical data, pathological findings, immunophenotyping (CD4, CD56, CD123), and treatment outcomes.
Main Results:
- Median age was 40 years; common sites of involvement included skin, lymph nodes, and bone marrow.
- Patients received multi-agent chemotherapy or radiotherapy; median progression-free survival was 8.6 months, overall survival was 15.1 months.
- All four patients with cutaneous BPDCN survived, contrasting with those without skin involvement.
Conclusions:
- BPDCN exhibits diverse clinical, histological, and immunophenotypical characteristics.
- Cutaneous involvement may serve as a positive prognostic factor in BPDCN, meriting further research.