Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Foster T, Bell D, et al. Blastic plasmacytoid dendritic cell neoplasm. Reference article, Radiopaedia.org (Accessed on 19 Mar 2024) https://doi.org/10.53347/rID-55923
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
It only represent a very small proportion (~0.44%) of all hematological malignancies 4. There may be a slight male predilection (may have a tendency to affect elderly males 7).
It often presents in the skin. Clinical course can be aggressive in adults and apparently milder in children. Some patient may have a leukemic presentation 5.
Histologically, the tumor cells may be either blastoid or pleomorphic. They express several markers of plasmacytoid dendritic cells which include:
CT
Pulmonary involvement
Has been described as interstitial opacities with a ground glass and reticular opacities 4.
Other involvement
Lymphadenopathy and hepatosplenomegaly may be present.
Nuclear medicine
FDG-PET
Skin lesions and lymph node metastases may appear hypermetabolic on 18F-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on 18F-FDG PET 4.
Treatment and prognosis
Chemotherapy is the preferred treatment, along with allogeneic stem cell transplantation. Patients usually have a poor outcome with a median survival reported around 12-14 months. Advanced stage and older age may imply poorer prognosis 4.
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7. Sapienza MR, Pileri A, Derenzini E, Melle F, Motta G, Fiori S, Calleri A, Pimpinelli N, Tabanelli V, Pileri S. Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects. (2019) Cancers. doi:10.3390/cancers11050595 - Pubmed
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