Blastic plasmacytoid dendritic cell neoplasm (BPDCN)

  • Method:
  • Anticoagulant:
  • Recommendation:
  • Method:
    Cytomorphology
  • Anticoagulant:
    EDTA
  • Recommendation:
    obligatory
  • Method:
    Immunophenotyping
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory
  • Method:
    Chromosome analysis
  • Anticoagulant:
    Heparin
  • Recommendation:
    obligatory
  • Method:
    FISH
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    facultative
  • Method:
    Molecular genetics
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory

BPDCN: Classification

The disease blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive, malignant disease with rapid systemic spread. BPDCN accounts for only about 0.4% of all hematologic neoplasms (Bueno et al. 2004, Pagano et al. 2013).

Initially, indolent courses with multiple skin lesions are most common. The skin manifestation is sometimes accompanied by lymph node involvement or bone marrow involvement. The extent of bone marrow infiltration varies greatly and results in cytopenias, especially thrombocytopenias (Feuillard et al. 2002, Pagano et al. 2013).

BPDCN is associated with clonal proliferation of immature precursors of plasmacytoid dendritic cells. Whether these are cells of the myeloid or lymphoid series has been a matter of controversy for years (Sapienza et al. 2019).

The diagnosis of BPDCN is associated with a previous or concurrent myelodysplastic or myelodysplastic/myeloproliferative neoplasia in 20-30% of patients. In addition, it may also develop after cytotoxic therapy for other malignancies. Differentially, BPDCN must be particularly distinguished from AML. Comparing the two diseases, overlapping characteristics regarding clinic, morphology, and immunophenotype may occur. Differentiation to proliferation of mature plasmacytoid dendritic cells (MPDCP) in patients with myeloid neoplasia should also be made. Here, the expression of CD34 together with pDC markers is considered a hallmark of AML with pDC differentiation (Khoury et al. 2022).

BPDCN: Diagnostic methods

BPDCN: Prognosis

The prognosis for BPDCN patients is unfavorable with a median overall survival of 8.7-24 months (Khoury et al. 2022). Response to first-line therapy, possibly followed by hematopoietic stem cell transplantation, plays an important role in prognosis. If complete remission is achieved with first-line therapy so that transplantation can be performed, overall survival is significantly prolonged (Garnache-Ottou et al. 2019).

BPDCN: Therapy

If possible, therapy should be initiated immediately after diagnosis. An overview of possible therapy algorithms is given in the Onkopedia guideline Blastic Plasmacytoid Dendritic Cell Neoplasia (BPDCN).

Status: February 2023

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