Real-world management of blastic plasmacytoid dendritic cell neoplasm at an academic center with a broad regional referral base

Nathan M Krah; Rasmus Hoeg; Paul J Shami; Shannon E Elf; Lauren E Lee; Naveen Pemmaraju; Ami B Patel

doi:10.1016/j.lrr.2025.100541

Real-world management of blastic plasmacytoid dendritic cell neoplasm at an academic center with a broad regional referral base

Leuk Res Rep. 2025 Aug 25:24:100541. doi: 10.1016/j.lrr.2025.100541. eCollection 2025.

Authors

Nathan M Krah¹, Rasmus Hoeg², Paul J Shami^{3

4}, Shannon E Elf^{3

4}, Lauren E Lee^{3

4}, Naveen Pemmaraju⁵, Ami B Patel^{3

4}

Affiliations

¹ Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
² Department of Internal Medicine, University of California, Davis Health, Sacramento, CA, USA.
³ Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
⁴ Huntsman Cancer Institute at the University of Utah, Salt Lake City, UT, USA.
⁵ Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Abstract

BPDCN is an aggressive myeloid malignancy characterized by unique expression of CD123, CD4, CD56, CD303, CD304, TCL1 and TCF4. The development of tagraxofusp, a CD123-directed cytotoxin, has revolutionized BPDCN treatment, especially for patients unfit for chemotherapy. While most patients respond to frontline tagraxofusp, there are challenges associated with treatment. In this case series, we describe our institutional experience treating BPDCN both pre- and post-tagraxofusp approval. We summarize six cases, the majority of which occurred in elderly patients, and highlight unique challenges of treating BPDCN at a center that serves a large rural/frontier area with variable access to specialty care.

Keywords: BPDCN; CD123; Myeloid neoplasm; Tagraxofusp.

Publication types

Case Reports