Treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN) with hyperfractionated cyclophosphamide, vincristine, adriamycin, and dexamethasone (HCVAD) in the first-line was associated with a higher complete response (CR) rate compared with other regimens, but not survival or duration of remission, according to the results of a retrospective study published in Blood Advances.

“These results suggest a continued important role for HCVAD-based chemotherapy in BPDCN, even in the modern targeted therapy era, with high CR rates in the frontline setting,” the authors wrote.

BPDCN is a highly aggressive cancer that frequently involves the central nervous system. Targeted therapy and hematopoietic stem cell transplant are typically not curative and central nervous system relapses are common. Prophylactic CNS-directed therapy is needed. The aim of this study was to evaluate the outcomes of patients with BPDCN treated with HCVAD.

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This single-center, retrospective study evaluated data from 100 patients treated for BPDCN between 1999 and 2020 and compared outcomes among patients who received HCVAD, SL-401, or other regimens.

Within the cohort, the median age at diagnosis was 61, 68, and 65 in the HCVAD, SL-401, and other regimen groups, respectively (P =.035). BPDCN most frequently involved bone marrow (70% to 75%), but was more common in the skin in the SL-401 group (92%) compared with the HCVAD (77%) and other regimen (64%) groups. The CNS and lymph nodes were more frequently involved in the HCVAD group (20% and 40%) compared with the SL-401 (3% and 16%) and other regimen (14% for both) groups (P =.07 and P =.022, respectively).

The CR rate was highest among patients treated with HCVAD at 80% compared with 59% and 43% among patients treated with SL-401 or other regimens, respectively (P =.01).

However, the higher response rate did not translate to improvements in survival or remission. Overall survival was similar between the groups, with a median of 28.3 months with HCVAD, 13.7 months with SL-401, and 22.8 months with other regimens (P =.41). The remission duration probability was also similar at 51%, 49%, and 38% in the HCVAD, SL-401, and other regimens groups, respectively (P =.455).

The authors concluded that, “further studies must establish the clinical activity, feasibility, and safety of doublet/triplet combinations of targeted therapies plus cytotoxic and the addition of CNS prophylaxis.”

Disclosures: Some of the study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.


Pemmaraju N, Wilson NR, Garcia-Manero G, et al. Characteristics and outcomes of patients with blastic plasmacytoid dendritic cell neoplasm treated with frontline HCVAD. Blood Adv. 2022;6:3027-3035. doi:10.1182/bloodadvances.2021006645