Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematologic 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 haematologic malignancies 4. There may be a slight male predilection.
It often presents in the skin. Clinical course can be aggressive in adults and apparently milder in children. Some patient may have a leukemic presenation 5.
Histologically, the tumor cells may be either blastoid or pleomorphic. They express several markers of plasmacytoid dendritic cells which include:
Lymphadenopathy and hepatosplenomegaly may be present.
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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