Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Article
  • Published:

Survival outcomes between haploidentical stem cell transplantation and chemotherapy for blastic plasmacytoid dendritic cell neoplasm

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive and rare hematological malignancy with poor clinical outcomes. Stem cell transplantation helps to achieve long-term survival in adults. However, the benefit of haploidentical stem cell transplantation (HID-SCT) versus chemotherapy is unclear with BPDCN. We retrospectively analyzed 32 patients diagnosed with BPDCN including 15 who underwent HID-SCT and 17 who only received chemotherapy. The median age was 52 (range, 19–78) years. The ratio of male/female was 2.2. Skin, bone marrow and lymph node were the most three common sites of disease involvement. Compared with the chemotherapy group, patients in the HID-SCT group had significantly better progression-free survival (PFS; median, 7 months versus not reached, P < 0.001) and overall survival (OS; median, 13 months versus not reached, P < 0.001). The 4-year rates for PFS and OS in transplant patients were 74% (95% Confidence Interval [CI], 47, 100%) and 93% (79, 100%), respectively, compared to 0 in non-transplant patients. In conclusion, our results demonstrated HID-SCT could provide long-term remissions in BPDCN patients.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Fig. 1: Survival outcomes of patients.
Fig. 2: Progression-free survival and overall survival according to haploidentical stem cell transplantation (HID-SCT).

Similar content being viewed by others

Data availability

All data generated or analyzed during this study are included in this published article.

References

  1. Roos-Weil D, Dietrich S, Boumendil A, Polge E, Bron D, Carreras E, et al. Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation. Blood. 2013;121:440–6.

    Article  CAS  PubMed  Google Scholar 

  2. Garnache-Ottou F, Vidal C, Biichlé S, Renosi F, Poret E, Pagadoy M, et al. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? Blood Adv. 2019;3:4238–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Zalmaï L, Viailly PJ, Biichle S, Cheok M, Soret L, Angelot-Delettre F, et al. Plasmacytoid dendritic cells proliferation associated with acute myeloid leukemia: phenotype profile and mutation landscape. Haematologica. 2021;106:3056–66.

    Article  PubMed  Google Scholar 

  4. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.

    Article  CAS  PubMed  Google Scholar 

  5. Frankel AE, Woo JH, Ahn C, Pemmaraju N, Medeiros BC, Carraway HE, et al. Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood. 2014;124:385–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Lu Y, Sun RJ, Zhang JP, Xu F, Du ZC, Tong GL, et al. Allogeneic hematopoietic stem cell transplantation with myeloablative conditioning regimen for blastic plasmacytoid dendritic cell neoplasm patients in complete remission: a single center study. Leuk lymphoma. 2022;63:3092–9.

    Article  CAS  PubMed  Google Scholar 

  7. Bashir Q, Milton DR, Popat UR, Kebriaei P, Hosing C, Khouri IF, et al. Allogeneic hematopoietic cell transplantation for patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN). Bone Marrow Transpl. 2022;57:51–6.

    Article  CAS  Google Scholar 

  8. Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98:239–46.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Cernan M, Szotkowski T, Hisemova M, Cetkovsky P, Sramkova L, Stary J, et al. Blastic plasmacytoid dendritic cell neoplasm: first retrospective study in the Czech Republic. Neoplasma. 2020;67:650–9.

    Article  CAS  PubMed  Google Scholar 

  10. Garnache-Ottou F, Feuillard J, Ferrand C, Biichle S, Trimoreau F, Seilles E, et al. Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol. 2009;145:624–36.

    Article  CAS  PubMed  Google Scholar 

  11. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114:937–51.

    Article  CAS  PubMed  Google Scholar 

  12. Yun S, Chan O, Kerr D, Vincelette ND, Idrees A, Mo Q, et al. Survival outcomes in blastic plasmacytoid dendritic cell neoplasm by first-line treatment and stem cell transplant. Blood Adv. 2020;4:3435–42.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Cuglievan B, Connors J, He J, Khazal S, Yedururi S, Dai J, et al. Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies. Leukemia. 2023;37:1767–78.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Pemmaraju N, Lane AA, Sweet KL, Stein AS, Vasu S, Blum W, et al. Tagraxofusp in blastic plasmacytoid dendritic-cell neoplasm. N Engl J Med. 2019;380:1628–37.

    Article  CAS  PubMed  Google Scholar 

  15. Montero J, Stephansky J, Cai T, Griffin GK, Cabal-Hierro L, Togami K, et al. Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax. Cancer Discov. 2017;7:156–64.

    Article  CAS  PubMed  Google Scholar 

  16. Alsidawi S, Westin GFM, Al-Kali A, Go RS. Blastic plasmacytoid dendritic cell neoplasm. a population-based analysis from the SEER and NCDB Databases. Blood. 2016;128:4789.

    Article  Google Scholar 

  17. Tsagarakis NJ, Kentrou NA, Papadimitriou KA, Pagoni M, Kokkini G, Papadaki H, et al. Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the Hellenic Dendritic Cell Leukemia Study Group. Leuk Res. 2010;34:438–46.

    Article  CAS  PubMed  Google Scholar 

  18. Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, et al. Long-term benefits of tagraxofusp for patients with blastic plasmacytoid dendritic cell neoplasm. J Clin Oncol: Off J Am Soc Clin Oncol 2022;40:3032–6.

    Article  CAS  Google Scholar 

  19. Jiang YL, Li Q, Yuan T, Jiang YY, Deng Q. Case report of anti-CD123 chimeric antigen receptor T-Cell therapy followed by radiotherapy for a recurrence of blastic plasmacytoid dendritic cell neoplasm after allogeneic hematopoietic stem cell transplantation. OncoTargets Ther. 2020;13:3425–30.

    Article  Google Scholar 

  20. Poussard M, Angelot-Delettre F, Deconinck E. Conventional therapeutics in BPDCN patients-do they still have a place in the era of targeted therapies? Cancers. 2022;14:3767.

  21. Liao C, Hu NX, Song H, Zhang JY, Shen DY, Xu XJ, et al. Pediatric blastic plasmacytoid dendritic cell neoplasm: report of four cases and review of literature. Int J Hematol. 2021;113:751–9.

    Article  CAS  PubMed  Google Scholar 

  22. Aoki T, Suzuki R, Kuwatsuka Y, Kako S, Fujimoto K, Taguchi J, et al. Long-term survival following autologous and allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm. Blood. 2015;125:3559–62.

    Article  CAS  PubMed  Google Scholar 

  23. Kharfan-Dabaja MA, Al Malki MM, Deotare U, Raj RV, El-Jurdi N, Majhail N, et al. Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study. Br J Haematol. 2017;179:781–9.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

RPG acknowledges support from the UK National Institute of Health Research (NIHR) Biomedical Research Centre. We thank all of the faculty members who participated in these studies.

Author information

Authors and Affiliations

Authors

Contributions

JL designed the study. JZ, FRW, SMY and YL analyzed the data. JZ, JL, RPG, YL and XDM prepared the typescript. YYZ, TZ, YQS, SZ, HZ, HC, PS, LW, JSJ and JW provided and collected the clinical data. All authors took responsibility for the content and agreed to submit for publication.

Corresponding author

Correspondence to Jin Lu.

Ethics declarations

Competing interests

RPG is a consultant to Antengene Biotech LLC; Medical Director, FFF Enterprises Inc.; A speaker for Janssen Pharma and Hengrui Pharma; Board of Directors: Russian Foundation for Cancer Research Support and Scientific Advisory Board, StemRad Ltd.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary information

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Zhou, J., Wang, F., Yang, S. et al. Survival outcomes between haploidentical stem cell transplantation and chemotherapy for blastic plasmacytoid dendritic cell neoplasm. Bone Marrow Transplant 60, 568–572 (2025). https://doi.org/10.1038/s41409-025-02528-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue date:

  • DOI: https://doi.org/10.1038/s41409-025-02528-y

Search

Quick links