open access

Vol 12, No 1 (2021)
Case report
Published online: 2021-06-13
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) — diagnostic and therapeutic challenge

Jarosław Wajs1, Małgorzata Lewandowska2, Przemysław Kluge3, Lucyna Drążek3, Dorota Jesionek-Kupnicka4
DOI: 10.5603/HCP.2021.0003
·
Hematology in Clinical Practice 2021;12(1):23-28.
Affiliations
  1. Hematology Unit, Mazowiecki Oncology Hospital in W ieliszew, Poland
  2. Department of Pathomorphology and Clinical Cytopathology, Medical University of Lodz, Poland
  3. Laboratory Diagnostics Department, Mazovian Oncology Hospital in Wieliszew, Poland
  4. Department of Pathology, Department of Oncology, Medical University of Lodz, Poland

open access

Vol 12, No 1 (2021)
CASE REPORTS
Published online: 2021-06-13

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy. The disease usually affects the skin, bone marrow, peripheral blood and less commonly lymph nodes. The actual incidence of BPDCN is currently unknown. The data presented in the literature most often relate to one cases or small groups of patients, rarely they are multicentre studies. Diagnosis of BPDCN is based on histopathological examination and immunohistochemical stains. The diversity of the clinical manifestations and the PBDCN immunophenotype is the cause of significant difficulties in making a diagnosis and can lead to diagnostic errors. The optimal treatment for patients with this cancer has not yet been established. Responses to various chemotherapy regimens are unsatisfactory. Recent literature has reported that bone marrow allograft or targeted therapy may improve treatment outcomes in this group of patients. The paper presents the case of a 75-year-old man with BPDCN diagnosis. Attention was paid to diagnostic difficulties in patients with BPDCN and the differentiation of this rare disease with other hematological malignancies was discussed. The need for a national register of BPDCN patients has been highlighted. This could contribute to expanding knowledge about this cancer and to the development of effective, standard therapeutic treatment.

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy. The disease usually affects the skin, bone marrow, peripheral blood and less commonly lymph nodes. The actual incidence of BPDCN is currently unknown. The data presented in the literature most often relate to one cases or small groups of patients, rarely they are multicentre studies. Diagnosis of BPDCN is based on histopathological examination and immunohistochemical stains. The diversity of the clinical manifestations and the PBDCN immunophenotype is the cause of significant difficulties in making a diagnosis and can lead to diagnostic errors. The optimal treatment for patients with this cancer has not yet been established. Responses to various chemotherapy regimens are unsatisfactory. Recent literature has reported that bone marrow allograft or targeted therapy may improve treatment outcomes in this group of patients. The paper presents the case of a 75-year-old man with BPDCN diagnosis. Attention was paid to diagnostic difficulties in patients with BPDCN and the differentiation of this rare disease with other hematological malignancies was discussed. The need for a national register of BPDCN patients has been highlighted. This could contribute to expanding knowledge about this cancer and to the development of effective, standard therapeutic treatment.
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Keywords

plasmacytoid dendritic cells, chemotherapy, immunohistochemistry

About this article
Title

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) — diagnostic and therapeutic challenge

Journal

Hematology in Clinical Practice

Issue

Vol 12, No 1 (2021)

Article type

Case report

Pages

23-28

Published online

2021-06-13

DOI

10.5603/HCP.2021.0003

Bibliographic record

Hematology in Clinical Practice 2021;12(1):23-28.

Keywords

plasmacytoid dendritic cells
chemotherapy
immunohistochemistry

Authors

Jarosław Wajs
Małgorzata Lewandowska
Przemysław Kluge
Lucyna Drążek
Dorota Jesionek-Kupnicka

References (27)
  1. Shi Y, Wang E. Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Arch Pathol Lab Med. 2014; 138(4): 564–569.
  2. Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 1994; 47(4): 278–282.
  3. Swerdlow SH, Campo E, Harris NL. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. International Agency for Research on Cancer, Lyon 2017.
  4. Julia F, Petrella T, Beylot-Barry M, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013; 169(3): 579–586.
  5. Deng W, Yang M, Kuang F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: a review of two cases. Mol Clin Oncol. 2017; 7(4): 709–715.
  6. Jegalian AG, Buxbaum NP, Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: diagnostic features and clinical implications. Haematologica. 2010; 95(11): 1873–1879.
  7. Nomburg J, Bullman S, Chung SS, et al. Comprehensive metagenomic analysis of blastic plasmacytoid dendritic cell neoplasm. Blood Adv. 2020; 4(6): 1006–1011.
  8. Feuillard J, Jacob MC, Valensi F, et al. Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood. 2002; 99(5): 1556–1563.
  9. Pagano L, Valentini CG, Pulsoni A, et al. GIMEMA-ALWP (Gruppo Italiano Malattie EMatologiche dell'Adulto, Acute Leukemia Working Party). Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013; 98(2): 239–246.
  10. Garnache-Ottou F, Vidal C, Biichlé S, et al. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? Blood Adv. 2019; 3(24): 4238–4251.
  11. Sapienza MR, Pileri A, Derenzini E, et al. Blastic plasmacytoid dendritic cell neoplasm: state of the art and prospects. Cancers (Basel). 2019; 11(5).
  12. Targoński Ł, Kwapisz D, Rymkiewicz G, et al. Nowotwór z blastycznych plazmocytoidnych komórek dendrytycznych (BPDCN)- obraz kliniczny, diagnostyka i leczenie w doświadczeniu jednego ośrodka. Onkol Prak Klin. 2013; 9(6): 230–238.
  13. Owczarczyk-Saczonek A, Sokołowska-Wojdyło M, Olszewska B, et al. Clinicopathologic retrospective analysis of blastic plasmacytoid dendritic cell neoplasms. Postepy Dermatol Alergol. 2018; 35(2): 128–138.
  14. Idrees A, Zhang X, Setoodeh R, et al. Clinical features and outcomes of 24 cases of blastic plasmacytoid dendritic cell neoplasm-single institutional experience. Blood. 2015; 126(23): 3755–3755.
  15. Alsidawi S, Westin G, Al-Kali A, et al. Blastic plasmacytoid dendritic cell neoplasm. a population-based analysis from the SEER and NCDB databases. Blood. 2016; 128(22): 4789–4789.
  16. Julia F, Dalle S, Duru G, et al. Blastic plasmacytoid dendritic cell neoplasms: clinico-immunohistochemical correlations in a series of 91 patients. Am J Surg Pathol. 2014; 38(5): 673–680.
  17. Martín-Martín L, López A, Vidriales B, et al. Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. Oncotarget. 2015; 6(22): 19204–19216.
  18. Rauh MJ, Rahman F, Good D, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Leuk Res. 2012; 36(1): 81–86.
  19. Herling M, Jones D. CD4+/CD56+ hematodermic tumor: the features of an evolving entity and its relationship to dendritic cells. Am J Clin Pathol. 2007; 127(5): 687–700.
  20. Martín-Martín L, Almeida J, Pomares H, et al. Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. Oncotarget. 2016; 7(9): 10174–10181.
  21. Marafioti T, Paterson JC, Ballabio E, et al. Novel markers of normal and neoplastic human plasmacytoid dendritic cells. Blood. 2008; 111(7): 3778–3792.
  22. Sukswai N, Aung PP, Yin CC, et al. Dual expression of TCF4 and CD123 is highly sensitive and specific for blastic plasmacytoid dendritic cell neoplasm. Am J Surg Pathol. 2019; 43(10): 1429–1437.
  23. Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016; 2016(1): 16–23.
  24. Roos-Weil D, Dietrich S, Boumendil A, et al. European Group for Blood and Marrow Transplantation Lymphoma, Pediatric Diseases, and Acute Leukemia Working Parties. 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(3): 440–446.
  25. Laribi K, Denizon N, Ghnaya H, et al. Blastic plasmacytoid dendritic cell neoplasm: the first report of two cases treated by 5-azacytidine. Eur J Haematol. 2014; 93(1): 81–85.
  26. Khwaja R, Daly A, Wong M, et al. Azacitidine in the treatment of blastic plasmacytoid dendritic cell neoplasm: a report of 3 cases. Leuk Lymphoma. 2016; 57(11): 2720–2722.
  27. Jen EY, Gao X, Li L, et al. FDA Approval Summary: Tagraxofusp-erzs For Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm. Clin Cancer Res. 2020; 26(3): 532–536.

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