Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
It only represent a wel small propotion (around 0.44%) of all haematological 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 leukaemic presenation 5.
Histologically, the tumour cells may be either blastoid or pleomorphic. The 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 F18-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on F18-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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