PD1/PD-L1 expression in blastic plasmacytoid dendritic cell neoplasm

Phyu P. Aung, Narittee Sukswai, Reza Nejati, Sanam Loghavi, Weina Chen, Carlos A. Torres-Cabala, C. Cameron Yin, Marina Konopleva, Xiaofeng Zheng, Jing Wang, Zhenya Tang, L. Jeffrey Medeiros, Victor G. Prieto, Naveen Pemmaraju, Joseph D. Khoury

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11 Scopus citations


Patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN) have poor outcomes despite intensive chemotherapy, underscoring the need for novel therapeutic approaches. The expression status of PD1/PD-L1 in BPDCN remains unknown. We evaluated PD1/PD-L1 by immunohistochemistry and RNAseq expression profiling in a cohort of BPDCN patients. The study group included 28 patients with a median age of 66.8 years (range, 22.8–86.7), 22 men and 6 women. PD-L1 expression was detected by immunohistochemistry in 10/21 (47.6%) cases. PD-L1 expression had a median H-score of 157. The H-score was ≥60 in 7 patients. PD-L1 protein levels (H-score) were proportional to normalized PD-L1 mRNA transcript levels (CD274 mRNA). In addition, high-level PD-L1 expression correlated with higher numbers of PD1-positive cells within BPDCN tumors. There was no correlation between clinicopathologic characteristics and PD-L1 expression status. Similarly, there was no significant difference in overall survival between patients with PD-L1-positive and PD-L1-negative BPDCN (median 12 vs. 23 month, respectively; p = 0.743). In conclusion, PD-L1 expression by tumor cells is detectable in a sizeable subset of patients with BPDCN, suggesting that exploration of the effectiveness of therapeutic inhibition of the PD1/PD-L1 axis in patients with refractory or progressive BPDCN is warranted.

Original languageEnglish (US)
Article number695
Issue number5
StatePublished - May 2019


  • Immune checkpoint regulation
  • Immunotherapy
  • PD-L1
  • RNA sequencing

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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