Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic malignancy originating from the precursor of the plasmacytoid dendritic cell. It is very rare and has only recently been recognized as a distinct entity. In this study, we report our experience of BPDCN to review the clinical, pathological features and treatment outcomes. A database at the Asan Medical Center was screened for all patients with BPDCN treated between 2000 and 2010. Seven patients were confirmed as BPDCN and included in this analysis. The median age of the patients was 40 years (range, 18–62 years), and four patients were male. Sites of disease involvement included the skin (n = 4), lymph node (n = 4), and peripheral blood/bone marrow (n = 2). Tumor cells were positive for CD4 (n = 5), CD56 (n = 6), and CD123 (n = 7). Six patients received multi-agent chemotherapy as first-line treatment, while one patient was given radiotherapy. The median progression-free survival was 8.6 months (range, 2.6–28.9 months) and overall survival was 15.1 months (range, 4.4–60.0 months) with a median follow-up period of 13.8 months (range, 1.9–29.9 months). Notably, all four patients with cutaneous involvement survived, whereas those without skin involvement succumbed to death, even though two of them were given salvage chemotherapy. In this study, patients with BPDCN showed various clinical, histological, and immunophenotypical features. Our experience warrants further examination of the prognostic significance of skin involvement in BPDCN.
Similar content being viewed by others
References
Herling M, Jones D (2007) CD4+/CD56+ hematodermic tumor: the features of an evolving entity and its relationship to dendritic cells. Am J Clin Pathol 127(5):687–700. doi:10.1309/fy6pk436nbk0ryd4
Facchetti FJD, Petrella T (2008) Blastic plasmacytoid dendritic cell neoplasm. WHO classification of tumors of haematopoietic and lymphoid tissues. IARC, Lyon
Chaperot L, Bendriss N, Manches O, Gressin R, Maynadie M, Trimoreau F, Orfeuvre H, Corront B, Feuillard J, Sotto JJ, Bensa JC, Briere F, Plumas J, Jacob MC (2001) Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood 97(10):3210–3217
Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, Meijer CJ, Courville P, Joly P, Grange F, De Muret A, Machet L, Dompmartin A, Bosq J, Durlach A, Bernard P, Dalac S, Dechelotte P, D’Incan M, Wechsler J, Teitell MA (2005) Blastic NK-cell lymphomas (agranular CD4+CD56+hematodermic neoplasms): a review. Am J Clin Pathol 123(5):662–675
Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L, Metze D, Fink-Puches R, Wiesner T, Cerroni L (2010) Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol 34(1):75–87. doi:10.1097/PAS.0b013e3181c5e26b
Argyrakos T, Rontogianni D, Karmiris T, Kapsimali V, Grigoriou E, Tsantekidou M, Naum C, Galani V, Pantelidaki C, Harhalakis N, Nikiforakis E, Kanavaros P (2004) Blastic natural killer (NK)-cell lymphoma: report of an unusual CD4 negative case and review of the CD4 negative neoplasms with blastic features in the literature. Leukemia & Lymphoma 45(10):2127–2133. doi:10.1080/10428190410001723232
Garnache-Ottou F, Chaperot L, Biichle S, Ferrand C, Remy-Martin JP, Deconinck E, de Tailly PD, Bulabois B, Poulet J, Kuhlein E, Jacob MC, Salaun V, Arock M, Drenou B, Schillinger F, Seilles E, Tiberghien P, Bensa JC, Plumas J, Saas P (2005) Expression of the myeloid-associated marker CD33 is not an exclusive factor for leukemic plasmacytoid dendritic cells. Blood 105(3):1256–1264. doi:10.1182/blood-2004-06-2416
Petrella T, Teitell MA, Spiekermann C, Meijer CJ, Franck F, Enache I (2004) A CD56-negative case of blastic natural killer-cell lymphoma (agranular CD4+/CD56+ haematodermic neoplasm). Br J Dermatol 150(1):174–176
Reimer P, Rudiger T, Kraemer D, Kunzmann V, Weissinger F, Zettl A, Konrad Muller-Hermelink H, Wilhelm M (2003) What is CD4+CD56+ malignancy and how should it be treated? Bone Marrow Transplantation 32(7):637–646. doi:10.1038/sj.bmt.1704215
Feuillard J, Jacob MC, Valensi F, Maynadie M, Gressin R, Chaperot L, Arnoulet C, Brignole-Baudouin F, Drenou B, Duchayne E, Falkenrodt A, Garand R, Homolle E, Husson B, Kuhlein E, Le Calvez G, Sainty D, Sotto MF, Trimoreau F, Bene MC (2002) Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood 99(5):1556–1563
Jegalian AG, Buxbaum NP, Facchetti F, Raffeld M, Pittaluga S, Wayne AS, Jaffe ES (2010) Blastic plasmacytoid dendritic cell neoplasm in children: diagnostic features and clinical implications. Haematol 95(11):1873–1879. doi:10.3324/haematol.2010.026179
Inoue D, Maruyama K, Aoki K, Nagano S, Maruoka H, Imai Y, Ito K, Ishikawa T, Takahashi T (2011) Blastic plasmacytoid dendritic cell neoplasm expressing the CD13 myeloid antigen. Acta Haematologica 126(2):122–128. doi:10.1159/000328180
Tsagarakis NJ, Kentrou NA, Papadimitriou KA, Pagoni M, Kokkini G, Papadaki H, Pappa V, Marinakis T, Anagnostopoulos NI, Vadikolia C, Anagnostopoulos A, Angelopoulou MK, Terpos E, Poziopoulos C, Anargyrou K, Rontogianni D, Papadaki T, Psarra A, Kontopidou FN, Skoumi D, Papadhimitriou SI, Paterakis G (2010) Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the hellenic dendritic cell leukemia study group. Leuk Res 34(4):438–446. doi:10.1016/j.leukres.2009.09.006
Garnache-Ottou F, Feuillard J, Ferrand C, Biichle S, Trimoreau F, Seilles E, Salaun V, Garand R, Lepelley P, Maynadie M, Kuhlein E, Deconinck E, Daliphard S, Chaperot L, Beseggio L, Foisseaud V, Macintyre E, Bene MC, Saas P, Jacob MC (2009) Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol 145(5):624–636. doi:10.1111/j.1365-2141.2009.07679.x
Petrella T, Facchetti F (2010) Tumoral aspects of plasmacytoid dendritic cells: what do we know in 2009? Autoimmun 43(3):210–214. doi:10.3109/08916930903510898
Dalle S, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P, Dompmartin A, D’Incan M, Maubec E, Grange F, Dereure O, Prey S, Barete S, Wetterwald M, Fraitag S, Petrella T (2010) Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol 162(1):74–79. doi:10.1111/j.1365-2133.2009.09373.x
Suzuki R, Nakamura S, Suzumiya J, Ichimura K, Ichikawa M, Ogata K, Kura Y, Aikawa K, Teshima H, Sako M, Kojima H, Nishio M, Yoshino T, Sugimori H, Kawa K, Oshimi K (2005) Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): prognostication and categorization according to anatomic sites of involvement. Cancer 104(5):1022–1031. doi:10.1002/cncr.21268
Li Y, Li Z, Lin HL, Chen XH, Li B (2011) Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: case report and review of the literature. Pathol Res Pract 207(1):55–59. doi:10.1016/j.prp.2010.05.008
Bekkenk MW, Jansen PM, Meijer CJ, Willemze R (2004) CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature. Ann Oncol 15(7):1097–1108. doi:10.1093/annonc/mdh268
Dijkman R, van Doorn R, Szuhai K, Willemze R, Vermeer MH, Tensen CP (2007) Gene-expression profiling and array-based CGH classify CD4+CD56+ hematodermic neoplasm and cutaneous myelomonocytic leukemia as distinct disease entities. Blood 109(4):1720–1727. doi:10.1182/blood-2006-04-018143
Jaye DL, Geigerman CM, Herling M, Eastburn K, Waller EK, Jones D (2006) Expression of the plasmacytoid dendritic cell marker BDCA-2 supports a spectrum of maturation among CD4+ CD56+ hematodermic neoplasms. Mod Pathol 19(12):1555–1562. doi:10.1038/modpathol.3800679
Conflict of interest
The authors declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Electronic supplementary material
Below is the link to the electronic supplementary material.
ESM 1
(PDF 152 kb)
Rights and permissions
About this article
Cite this article
An, H.J., Yoon, D.H., Kim, S. et al. Blastic plasmacytoid dendritic cell neoplasm: a single-center experience. Ann Hematol 92, 351–356 (2013). https://doi.org/10.1007/s00277-012-1614-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00277-012-1614-z