Blastic plasmacytoid dendritic cell neoplasm

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 leukaemia.

It only represent a very small proportion (~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. They express several markers of plasmacytoid dendritic cells which include:

  • CD123
  • TCL1
  • BDCA2
  • CD2AP

Has been described as interstitial opacities with a ground glass and reticular opacities 4.

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.

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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Article information

rID: 55923
Synonyms or Alternate Spellings:
  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
  • blastic natural-killer lymphoma
  • agranular CD4+ natural killer cell leukaemia

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