Abstract
Background: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy arising from plasmacytoid dendritic cells. It commonly presents as skin lesions with or without bone marrow and soft tissue involvement. Limited retrospective data have shown durable remissions after both autologous (auto) or allogeneic (allo) hematopoietic cell transplantation (HCT). Aims: To evaluate progression-free survival (PFS) and overall survival (OS) after and auto or allo-HCT. Methods: In this retrospective single-center analysis, we evaluated the outcomes of BPDCN patients who received either an auto- or allo-HCT between September 2000 and January 2019 at our institution. Results: We identified 24 consecutive patients who received an auto-HCT (n = 10) or allo-HCT (n = 14) for BPDCN. Skin involvement, with or without other organs, was seen in 16 (67%), BM involvement, with or without soft tissue, in 5 (21%), and other organs in 3 (13%) patients. Patient characteristics are summarized in the attached table. In general, we used auto-HCT for older patients and those with skin-only involvement. Median age at HCT was 52 (range: 18–79) years for all patients. Median (range) follow up in auto-HCT and allo-HCT patients was 6.2 (0.2–37.6) and 8.4 (1.1–76.1) months, respectively (p = 0.52). One-hundred day and 1-year non-relapse mortality (NRM) in auto-HCT was 21% and 35%, and in allo-HCT was 7% and 32%, respectively (p = 0.58). Two of the 14 (14%) allo patients and 2 of the 10 (20%) auto patients progressed after HCT (p = 1.00). Two-year PFS was 28% (4%, 60%) and 47% (95% confidence interval = 17%, 73%) in auto and allo-HCT, respectively (p = 0.34). Two-year OS was 26% (4%, 57%) and 56% (24%, 80%) in auto and allo-HCT, respectively (p = 0.33). In patients transplanted <2015 vs. ≥ 2015, 2-year PFS was 13% vs. 54% (p = 0.009) and 2 year OS was 13% and 68% (p = 0.017).Summary/Conclusion: These results demonstrate the efficacy of both auto and allo-HCT in BPDCN, with a significant improvement in the outcome since 2015. Prospective studies are needed to better define the role of HCT in BPDCN.
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