Quick complete response achievement with venetoclax and azacitidine in a case of relapsed disseminated blastic plasmacytoid dendritic cell neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and extremely aggressive myeloid malignancy arising from dendritic cell (DC) precursors typically affecting skin and/or bone marrow. Despite excellent complete response (CR) rates with induction chemotherapy, the disease has a marked tendency to relapse and become chemoresistant, with an overall dismal outcome. Frontline treatment with acute myeloid leukemia-like and acute lymphoblastic leukemia-like regimens resulted in disease-free and overall survival advantage, although allogeneic stem cell transplantation appears to be the only therapeutic approach with potential for disease eradication and long-term survivorship . Data on efficacy of specific therapeutic regimens in the relapsed/refractory (R/R) setting are lacking, mainly because of the rarity of this disease entity. Conventional chemotherapy seems to provide only limited and transient clinical benefit . Recently, CD123-directed cytotoxin...
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Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.Informed consent.
Informed consent was obtained from all individual participants included in the study.
- 4.Montero J, Stephansky J, Cai T, Griffin GK, Cabal-Hierro L, Togami K, Hogdal LJ, Galinsky I, Morgan EA, Aster JC, Davids MS, LeBoeuf NR, Stone RM, Konopleva M, Pemmaraju N, Letai A, Lane AA (2017 February) Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL-2 and sensitive to venetoclax. Cancer Discov 7(2):156–164CrossRefGoogle Scholar