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Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents
Shunan Wang1, Xingtong Wang1, Mingsuo Liu1
1Department of Hematology, The First Hospital of Jilin University, No. 71 Xinmin Street, Chaoyang District, Changchun, 130021, Jilin, People's Republic of China.
Annals of Hematology
|February 19, 2018
Summary
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare cancer. While chemotherapy and stem cell transplants offer limited survival, novel targeted therapies show promise for future treatment.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy.
- It primarily affects elderly individuals, often presenting with skin lesions and bone marrow involvement.
- Characteristic immunophenotype includes CD4+ and CD56+ co-expression, lacking lymphoid or myeloid markers.
Purpose of the Study:
- To review the current understanding of BPDCN.
- To discuss existing therapeutic strategies and their limitations.
- To explore emerging targeted therapies for BPDCN.
Main Methods:
- Literature review of BPDCN.
- Analysis of clinical presentation and diagnostic criteria.
- Evaluation of treatment outcomes and survival data.
- Assessment of novel therapeutic targets and early clinical trial results.
Main Results:
- BPDCN exhibits high responsiveness to chemotherapy but short median event-free survival.
- Allogeneic stem cell transplantation may improve prognosis but has a high relapse rate.
- No specific targeted agents are currently approved for BPDCN.
Conclusions:
- Optimal therapeutic strategy for BPDCN remains undefined.
- Advances in understanding BPDCN pathobiology reveal novel therapeutic targets.
- Novel targeted therapies hold potential to improve future outcomes for BPDCN patients.
Keywords:
Blastic plasmacytoid dendritic cell neoplasmCentral nervous system prophylaxisHematopoietic stem cell transplantationNovel targeted agentsMore Related Videos
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