Low-dose venetoclax combined with azacitidine for blastic plasmacytoid dendritic cell neoplasm: a case report and literature review

Ann Hematol. 2024 Mar;103(3):999-1005. doi: 10.1007/s00277-024-05633-y. Epub 2024 Jan 29.

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that is highly aggressive with a poor prognosis. There is no standard treatment for BPDCN. Although conventional chemotherapies are usually sensitive in the initial therapy, relapse and drug resistance are inevitable within a short duration. Targeted therapies have enlightened new prospects for the treatment of BPDCN, especially for those in a frail state and intolerable to standard chemotherapies or hematopoietic stem cell transplantation. Here, we report an 82-year-old man diagnosed with cutaneous-limited BPDCN. Considering the old age and limited involvement of the tumor, we reduced the dosage of venetoclax. His skin lesions subsided significantly after 1 cycle of azacytidine (100 mg d1-7) combined with reduced doses of venetoclax (200 mg d1-14). The reduction in the dose of venetoclax avoided severe myelosuppression while achieving satisfactory outcomes. The patient received 2 cycles of therapy with no skin lesions re-occurred for 7 months before relapsing.

Keywords: Azacitidine; Blastic plasmacytoid dendritic cell neoplasm; Venetoclax.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Azacitidine / therapeutic use
  • Bridged Bicyclo Compounds, Heterocyclic*
  • Dendritic Cells / pathology
  • Hematologic Neoplasms* / therapy
  • Humans
  • Male
  • Myeloproliferative Disorders* / pathology
  • Neoplasm Recurrence, Local / drug therapy
  • Skin Neoplasms* / pathology
  • Sulfonamides*

Substances

  • Azacitidine
  • venetoclax
  • Sulfonamides
  • Bridged Bicyclo Compounds, Heterocyclic