Different Presentations and Different Treatment Options in Blastic Plasmacytoid Dendritic Cell Neoplasms: A Case Series
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Abstract
Blastic plasmacytoid dendritic cell neoplasms (BPDCN) are rare, aggressive hematologic neoplasms. Awareness about this neoplasm has increased after it was defined as a clonal plasmacytoid dendritic cell disease under histiocytic/dendritic cell neoplasms in the World Health Organization 2022 classification of myeloid and Histiocytic/Dendritic Neoplasms1. Therapies include chemotherapy or immunotherapy2-4 though stem cell transplantation (SCT) is the best consolidative approach in eligible patients5. Here, we present one intensive therapy-ineligible and two intensive-therapy-eligible patients with different presentations of BPDCN.
1. Khoury JD, Solary E, Abla O, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia. 2022;36(7):1720-1748.
2. Adimora IJ, Wilson NR, Pemmaraju N. Blastic plasmacytoid dendritic cell neoplasm (BPDCN): A promising future in the era of targeted therapeutics. Cancer. 2022;128(16):3019-26.
3. Haddadin M, Taylor J. Chemotherapy Options for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):539-52.
4. Lane AA. Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):589-600.
5. Kharfan-Dabaja MA, Cherry M. Hematopoietic Cell Transplant for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):621-9.
6. Zhang Y, Sokol L. Clinical Insights into the Management of Blastic Plasmacytoid Dendritic Cell Neoplasm. Cancer Manag Res. 2022;14:2107-17.
7. Sakamoto K, Takeuchi K. Cytogenetics of Blastic Plasmacytoid Dendritic Cell Neoplasm: Chromosomal Rearrangements and DNA Copy-Number Alterations. Hematol Oncol Clin North Am. 2020;34(3):523-38.
8. Deconinck E, Petrella T, Garnache Ottou F. Blastic Plasmacytoid Dendritic Cell Neoplasm: Clinical Presentation and Diagnosis. Hematol Oncol Clin North Am. 2020;34(3):491-500.
9. El Hussein S, Wang W, Wang SA, et al. Blastic plasmacytoid dendritic cell neoplasm with history of myeloma and concomitant acute undifferentiated leukemia: Illustration of morphologic and immunophenotypic challenges of an emerging phenomenon. Leuk Lymphoma. 2021;62(13):3296-3299.
10. Pemmaraju N, Kantarjian HM, Khoury JD, et al. Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Commonly Presents in the Setting of Prior or Concomitant Hematologic Malignancies (PCHM): Patient Characteristics and Outcomes in the Rapidly Evolving Modern Targeted Therapy Era. Blood. 2019; 134 (Supplement_1): 2723.
11. Yun S, Chan O, Kerr D, et al. Survival outcomes in blastic plasmacytoid dendritic cell neoplasm by first-line treatment and stem cell transplant. Blood Adv. 2020;4(14):3435-42.
12. Marmouset V, Joris M, Merlusca L, et al. The lenalidomide/bortezomib/dexamethasone regimen for the treatment of blastic plasmacytoid dendritic cell neoplasm. Hematol Oncol. 2019;37(4):487-489.
13. Iversen KF, Holdgaard PC, Preiss B, et al. Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm. A single-case report. Haematologica. 2019;104(9):e432-e433.
14. Mirgh S, Sharma A, Folbs B, et al. Daratumumab-based therapy after prior Azacytidine-Venetoclax in an octagenerian female with BPDCN (blastic plasmacytoid dendritic cell neoplasm) - a new perspective. Leuk Lymphoma. 2021;62(12):3039-3042.
15. Bétrian S, Guenounou S, Luquet I, et al. Bendamustine for relapsed blastic plasmacytoid dendritic cell leukaemia. Hematol Oncol. 2017;35(2):252-255.
16. Wang W, Khoury JD, Miranda RN, et al. Immunophenotypic characterization of reactive and neoplastic plasmacytoid dendritic cells permits establishment of a 10-color flow cytometric panel for initial workup and residual disease evaluation of blastic plasmacytoid dendritic cell neoplasm. Haematologica. 2021;106(4):1047-55.
17. Kharfan-Dabaja MA, Reljic T, Murthy HS, et al. Allogeneic hematopoietic cell transplantation is an effective treatment for blastic plasmacytoid dendritic cell neoplasm in first complete remission: systematic review and meta-analysis. Clin Lymphoma Myeloma Leuk. 2018;18(11):703-709.e1.
18. Taylor J, Haddadin M, Upadhyay VA, et al. Multicenter analysis of outcomes in blastic plasmacytoid dendritic cell neoplasm offers a pretargeted therapy benchmark. Blood. 2019;134(8):678-687.
2. Adimora IJ, Wilson NR, Pemmaraju N. Blastic plasmacytoid dendritic cell neoplasm (BPDCN): A promising future in the era of targeted therapeutics. Cancer. 2022;128(16):3019-26.
3. Haddadin M, Taylor J. Chemotherapy Options for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):539-52.
4. Lane AA. Novel Therapies for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):589-600.
5. Kharfan-Dabaja MA, Cherry M. Hematopoietic Cell Transplant for Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34(3):621-9.
6. Zhang Y, Sokol L. Clinical Insights into the Management of Blastic Plasmacytoid Dendritic Cell Neoplasm. Cancer Manag Res. 2022;14:2107-17.
7. Sakamoto K, Takeuchi K. Cytogenetics of Blastic Plasmacytoid Dendritic Cell Neoplasm: Chromosomal Rearrangements and DNA Copy-Number Alterations. Hematol Oncol Clin North Am. 2020;34(3):523-38.
8. Deconinck E, Petrella T, Garnache Ottou F. Blastic Plasmacytoid Dendritic Cell Neoplasm: Clinical Presentation and Diagnosis. Hematol Oncol Clin North Am. 2020;34(3):491-500.
9. El Hussein S, Wang W, Wang SA, et al. Blastic plasmacytoid dendritic cell neoplasm with history of myeloma and concomitant acute undifferentiated leukemia: Illustration of morphologic and immunophenotypic challenges of an emerging phenomenon. Leuk Lymphoma. 2021;62(13):3296-3299.
10. Pemmaraju N, Kantarjian HM, Khoury JD, et al. Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Commonly Presents in the Setting of Prior or Concomitant Hematologic Malignancies (PCHM): Patient Characteristics and Outcomes in the Rapidly Evolving Modern Targeted Therapy Era. Blood. 2019; 134 (Supplement_1): 2723.
11. Yun S, Chan O, Kerr D, et al. Survival outcomes in blastic plasmacytoid dendritic cell neoplasm by first-line treatment and stem cell transplant. Blood Adv. 2020;4(14):3435-42.
12. Marmouset V, Joris M, Merlusca L, et al. The lenalidomide/bortezomib/dexamethasone regimen for the treatment of blastic plasmacytoid dendritic cell neoplasm. Hematol Oncol. 2019;37(4):487-489.
13. Iversen KF, Holdgaard PC, Preiss B, et al. Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm. A single-case report. Haematologica. 2019;104(9):e432-e433.
14. Mirgh S, Sharma A, Folbs B, et al. Daratumumab-based therapy after prior Azacytidine-Venetoclax in an octagenerian female with BPDCN (blastic plasmacytoid dendritic cell neoplasm) - a new perspective. Leuk Lymphoma. 2021;62(12):3039-3042.
15. Bétrian S, Guenounou S, Luquet I, et al. Bendamustine for relapsed blastic plasmacytoid dendritic cell leukaemia. Hematol Oncol. 2017;35(2):252-255.
16. Wang W, Khoury JD, Miranda RN, et al. Immunophenotypic characterization of reactive and neoplastic plasmacytoid dendritic cells permits establishment of a 10-color flow cytometric panel for initial workup and residual disease evaluation of blastic plasmacytoid dendritic cell neoplasm. Haematologica. 2021;106(4):1047-55.
17. Kharfan-Dabaja MA, Reljic T, Murthy HS, et al. Allogeneic hematopoietic cell transplantation is an effective treatment for blastic plasmacytoid dendritic cell neoplasm in first complete remission: systematic review and meta-analysis. Clin Lymphoma Myeloma Leuk. 2018;18(11):703-709.e1.
18. Taylor J, Haddadin M, Upadhyay VA, et al. Multicenter analysis of outcomes in blastic plasmacytoid dendritic cell neoplasm offers a pretargeted therapy benchmark. Blood. 2019;134(8):678-687.
| Files | ||
| Issue | Vol 19 No 1 (2025) | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/ijhoscr.v19i1.17829 | |
| Keywords | ||
| Bendamustine; Blastic plasmacytoid dendritic cell neoplasm; Chemotherapy; Minimal residual disease; Stem cell transplantation | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Cengiz E, Aydin M, Gunes A, Ceran F, Dagdas S, Ozet G. Different Presentations and Different Treatment Options in Blastic Plasmacytoid Dendritic Cell Neoplasms: A Case Series. Int J Hematol Oncol Stem Cell Res. 2025;19(1):81-85.
