Oncohematology

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)

A clinical case of BPDCN mimicking lymphoma: cell morphology in blood smear, immunophenotyping (CD56, CD123), and molecular diagnostics.

Blood smear with atypical cells and flow cytometry in BPDCN
Blood smear with atypical cells and flow cytometry in BPDCN

🔬 Rare Diagnosis: How a “Lymphoma Mimic” Confused Doctors?

A 60-year-old man was admitted to the hospital with hematomas and enlarged lymph nodes. Tests showed abnormal “lymphocytes” (86% of all cells), initially leading to a suspicion of lymphoproliferative disease.

🔎 What was found? Upon detailed examination:

  • A blood smear revealed atypical cells — some resembled mature lymphocytes, others had vacuoles or grayish cytoplasm.
  • However, flow cytometry (a precise cell analysis method) identified not lymphoma, but a rare blastic plasmacytoid dendritic cell neoplasm (BPDCN).

🧬 Why is this important?

  • BPDCN is rare and often “mimics” other diseases.
  • In this case, almost all cells looked lymphoid — a rare finding!
  • Key to the diagnosis were specific markers (CD56, CD123, HLA-DR) and mutations in TET2, ASXL1, NF1 genes.

💡 Conclusion Even with a “typical” picture, it is important to use additional methods — otherwise, a rare but aggressive tumor can be missed.

Source: Ravel-Chapuis R. et al. American Journal of Hematology. 2025;100:1432–1433.