Advertisement

Annals of Hematology

, Volume 97, Issue 4, pp 563–572 | Cite as

Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents

  • Shunan Wang
  • Xingtong Wang
  • Mingsuo Liu
  • Ou Bai
Review Article

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematopoietic malignancy mainly affecting elderly patients. Most patients present with asymptomatic skin lesions as the first symptom and has a high frequency of bone marrow involvement. BPDCN is typically characterized by CD4+ and CD 56+ co-expression without common lymphoid or myeloid lineage markers. There is no consensus on the optimal therapeutic strategy for BPDCN. It is highly responsive to chemotherapy but the median event-free survival is very short. Allogeneic stem cell transplantation may improve the prognosis of BPDCN but the rate of relapse is still high. There are no specific targeted agents approved for patients with BPDCN, but advances in the understanding of the pathobiology of BPDCN and the results of early clinical studies have revealed novel targets and potentially effective agents. Novel targeted therapies may improve outcomes for patients with BPDCN in the future.

Keywords

Blastic plasmacytoid dendritic cell neoplasm Hematopoietic stem cell transplantation Central nervous system prophylaxis Novel targeted agents 

Notes

Funding information

This work was supported by the development and reform commission of Jilin province, research and development of individualized information management system on malignancies with high morbidity (2014Y090).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

References

  1. 1.
    Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (2008) WHO classification of tumours of hematopoietic and lymphoid tissues, 4th edn. IARC Press, LyonGoogle Scholar
  2. 2.
    Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127(20):2391–2405.  https://doi.org/10.1182/blood-2016-03-643544 CrossRefPubMedGoogle Scholar
  3. 3.
    Julia F, Dalle S, Duru G, Balme B, Vergier B, Ortonne N, Vignon-Pennamen MD, Costes-Martineau V, Lamant L, Dalac S, Delattre C, Déchelotte P, Courville P, Carlotti A, De Muret A, Fraitag S, Levy A, Mitchell A, Petrella T (2014) Blastic plasmacytoid dendritic cell neoplasms: clinico-immunohistochemical correlations in a series of 91 patients. Am J Surg Pathol 38(5):673–680.  https://doi.org/10.1097/PAS.0000000000000156 CrossRefPubMedGoogle Scholar
  4. 4.
    Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, Lunghi M, Pica G, Onida F, Cattaneo C, Piccaluga PP, Di Bona E, Todisco E, Musto P, Spadea A, D'Arco A, Pileri S, Leone G, Amadori S, Facchetti F, GIMEMA-ALWP (Gruppo Italiano Malattie EMatologiche dell'Adulto, Acute Leukemia Working Party) (2013) Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 98(2):239–246.  https://doi.org/10.3324/haematol.2012.072645 CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Julia F, Petrella T, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P, Dereure O, Wetterwald M, d'Incan M, Grange F, Cornillon J, Tertian G, Maubec E, Saiag P, Barete S, Templier I, Aubin F, Dalle S (2013) Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol 169(3):579–586.  https://doi.org/10.1111/bjd.12412 CrossRefPubMedGoogle Scholar
  6. 6.
    Bueno C, Almeida J, Lucio P, Marco J, Garcia R, de Pablos JM, Parreira A, Ramos F, Ruiz-Cabello F, Suarez-Vilela D, San Miguel JF, Orfao A (2004) Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica 89(1):58–69PubMedGoogle Scholar
  7. 7.
    Sullivan JM, Rizzieri DA (2016) Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematol Am Soc Hematol Educ Program 2016(1):16–23.  https://doi.org/10.1182/asheducation-2016.1.16 Google Scholar
  8. 8.
    Cmds BR, Cjd SJ, Lac L, Mjr GA, Ns A, Afl C, Ms M, Vmds G, Amc SB, Jas Q, Nb DS (2017) Blastic plasmacytoid dendritic cell neoplasm with pulmonary involvement and atypical skin lesion. Am J Case Rep 18:692–695.  https://doi.org/10.12659/AJCR.903059 CrossRefGoogle Scholar
  9. 9.
    Rauh MJ, Rahman F, Good D, Silverman J, Brennan MK, Dimov N, Liesveld J, Ryan DH, Burack WR, Bennett JM (2012) Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: case series and literature review. Leuk Res 36(1):81–86.  https://doi.org/10.1016/j.leukres.2011.07.033 CrossRefPubMedGoogle Scholar
  10. 10.
    Amitay-Laish I, Sundram U, Hoppe RT, Hodak E, Medeiros BC, Kim YH (2017) Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: a subset with possible durable remission without transplantation. JAAD Case Rep 3(4):310–315.  https://doi.org/10.1016/j.jdcr.2017.03.015 CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Kong SH, Han SH, Suh HS, Choi YS (2017) Blastic plasmacytoid dendritic cell neoplasm presenting as erythematous nodules with gallbladder involvement. Ann Dermatol 29(4):501–503.  https://doi.org/10.5021/ad.2017.29.4.501 CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    García-Colmenero L, Gallardo F, Salar A, Alvarez-Larrán A, Colomo L, Pujol RM (2016) Blastic plasmacytoid dendritic cell neoplasm associated with dystrophic Xanthomatosis after chemotherapy. Acta Derm Venereol 97(2):293–294.  https://doi.org/10.2340/00015555-2515 CrossRefGoogle Scholar
  13. 13.
    Rajkumari BD, Munikoty V, Sreedharanunni S, Jain R, Sachdeva MUS, Varma N (2017) Childhood blastic plasmacytoid dendritic cell neoplasm mimicking acute rheumatic fever: report of an unusual clinical presentation and review of literature. J Pediatr Hematol Oncol 7:709–715.  https://doi.org/10.1097/MPH.0000000000000951 Google Scholar
  14. 14.
    Lourdes MM, Julia A, Helena P, Eva GB, Pilar B, Teresa G, Cecilia H, Jose AQ, Elena PC, Violeta M, Natalia A, Carlota C, Rodolfo A, María DC, Alberto O (2016) Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. Oncotarget 7(9):10174–10181.  https://doi.org/10.18632/oncotarget.7101 Google Scholar
  15. 15.
    Qubty WF, Al-Kali A, Jones LK Jr (2015) Leukemic polyradiculopathy due to blastic plasmacytoid dendritic cell neoplasm. JAMA Neurol 72(8):938–939.  https://doi.org/10.1001/jamaneurol.2015.0830 CrossRefPubMedGoogle Scholar
  16. 16.
    Tzankov A, Hebeda K, Kremer M, Leguit R, Orazi A, van der Walt J, Gianelli U (2017) Plasmacytoid dendritic cell proliferations and neoplasms involving the bone marrow: summary of the workshop cases submitted to the 18th meeting of the European Association for Haematopathology (EAHP) organized by the European bone marrow working group, Basel 2016. Ann Hematol 96(5):765–777.  https://doi.org/10.1007/s00277-017-2947-4 CrossRefPubMedGoogle Scholar
  17. 17.
    Riaz W, Zhang L, Horna P, Sokol L (2014) Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control 21(4):279–289.  https://doi.org/10.1177/107327481402100404 CrossRefPubMedGoogle Scholar
  18. 18.
    Lourdes MM, Antonio L, Belen V, Maria DC, Antonio SR, Silvia IF, Margarida L, Sergio A, Berta V, Pilar M, Ana F, Jorge C, Francisco RC, Josefa MB, Amparo S, Neus V, Alberto O, Julia A (2015) Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. Oncotarget 6(22):19204–19216.  https://doi.org/10.18632/oncotarget.4146 Google Scholar
  19. 19.
    Yigit N, Suarez LF, Roth LG, Orazi A, Tam W (2017) CD4-negative variant of cutaneous blastic plasmacytoid dendritic cell neoplasm with a novel PBRM1 mutation in an 11-year-old girl. Am J Clin Pathol 147(5):453–460.  https://doi.org/10.1093/ajcp/aqx012 CrossRefPubMedGoogle Scholar
  20. 20.
    Takiuchi Y, Maruoka H, Aoki K, Kato A, Ono Y, Nagano S, Arima H, Inoue D, Mori M, Tabata S, Yanagita S, Matsushita A, Nishio M, Imai Y, Imai Y, Ito K, Fujita H, Kadowaki N, Ishikawa T, Takahashi T (2012) Leukemic manifestation of blastic plasmacytoid dendritic cell neoplasm lacking skin lesion: a borderline case between acute monocytic leukemia. J Clin Exp Hematop 52(2):107–111.  https://doi.org/10.3960/jslrt.52.107 CrossRefPubMedGoogle Scholar
  21. 21.
    Kim HS, Kim HJ, Kim SH, Choi JY, Ko YH, Kim WS, Jung CW, Kim SJ (2017) Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell neoplasm: a single-center experience in Korea. Korean J Intern Med 32(5):890–899.  https://doi.org/10.3904/kjim.2015.406 CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Montes-Moreno S, Ramos-Medina R, Martínez-López A, Barrionuevo Cornejo C, Parra Cubillos A, Quintana-Truyenque S, Rodriguez Pinilla SM, Pajares R, Sanchez-Verde L, Martinez-Torrecuadrada J, Roncador G, Piris MA (2013) SPIB, a novel immunohistochemical marker for human blastic plasmacytoid dendritic cell neoplasms: characterization of its expression in major hematolymphoid neoplasms. Blood 121(4):643–647.  https://doi.org/10.1182/blood-2012-08-447599 CrossRefPubMedGoogle Scholar
  23. 23.
    Salva KA, Haemel AK, Pincus LB, Liu J, Sundram U, Guitart J, Longley BJ, Wood GS (2014) Expression of CD31/PECAM-1 (platelet endothelial cell adhesion molecule 1) by blastic plasmacytoid dendritic cell neoplasms. JAMA Dermatol 150(1):73–76.  https://doi.org/10.1001/jamadermatol.2013.7141 CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Garnache-Ottou F, Feuillard J, Ferrand C, Biichle S, Trimoreau F, Seilles E, Salaun V, Garand R, Lepelley P, Maynadié M, Kuhlein E, Deconinck E, Daliphard S, Chaperot L, Beseggio L, Foisseaud V, Macintyre E, Bene MC, Saas P, Jacob MC, GOELAMS and GEIL study (2009) Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol 145(5):624–636.  https://doi.org/10.1111/j.1365-2141.2009.07679.x CrossRefPubMedGoogle Scholar
  25. 25.
    Lucioni M, Novara F, Fiandrino G, Riboni R, Fanoni D, Arra M, Venegoni L, Nicola M, Dallera E, Arcaini L, Onida F, Vezzoli P, Travaglino E, Boveri E, Zuffardi O, Paulli M, Berti E (2011) Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood 118(17):4591–4594.  https://doi.org/10.1182/blood-2011-03-337501 CrossRefPubMedGoogle Scholar
  26. 26.
    Leroux D, Mugneret F, Callanan M, Radford-Weiss I, Dastugue N, Feuillard J, Le Mée F, Plessis G, Talmant P, Gachard N, Uettwiller F, Pages MP, Mozziconacci MJ, Eclache V, Sibille C, Avet-Loiseau H, Lafage-Pochitaloff M (2002) CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique. Blood 99(11):4154–4159.  https://doi.org/10.1182/blood.V99.11.4154 CrossRefPubMedGoogle Scholar
  27. 27.
    Wiesner T, Obenauf AC, Cota C, Fried I, Speicher MR, Cerroni L (2010) Alterations of the cell-cycle inhibitors p27(KIP1) and p16(INK4a) are frequent in blastic plasmacytoid dendritic cell neoplasms. J Invest Dermatol 130(4):1152–1157.  https://doi.org/10.1038/jid.2009.369 CrossRefPubMedGoogle Scholar
  28. 28.
    Nakamura Y, Kayano H, Kakegawa E, Miyazaki H, Nagai T, Uchida Y, Ito Y, Wakimoto N, Mori S, Bessho M (2015) Identification of SUPT3H as a novel 8q24/MYC partner in blastic plasmacytoid dendritic cell neoplasm with t (6;8) (p21; q24) translocation. Blood Cancer J 5(4):e301.  https://doi.org/10.1038/bcj.2015.26 CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Cao Q, Liu F, Niu G, Xue L, Han A (2014) Blastic plasmacytoid dendritic cell neoplasm with EWSR1 gene rearrangement. J Clin Pathol 67(1):90–92.  https://doi.org/10.1136/jclinpath-2013-201792 CrossRefPubMedGoogle Scholar
  30. 30.
    Gao NA, Wang XX, Sun JR, Yu WZ, Guo NJ (2015) Blastic plasmacytoid dendritic cell neoplasm with leukemic manifestation and ETV6 gene rearrangement: a case report. Exp Ther Med 9(4):1109–1112.  https://doi.org/10.3892/etm.2015.2236 CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Tokuda K, Eguchi-Ishimae M, Yagi C, Kawabe M, Moritani K, Niiya T, Tauchi H, Ishii E, Eguchi M (2014) CLTC-ALK fusion as a primary event in congenital blastic plasmacytoid dendritic cell neoplasm. Genes Chromosomes Cancer 53(1):78–89.  https://doi.org/10.1002/gcc.22119 CrossRefPubMedGoogle Scholar
  32. 32.
    Yang N, Huh J, Chung WS, Cho MS, Ryu KH, Chung HS (2015) KMT2A (MLL)-MLLT1, rearrangement in blastic plasmacytoid dendritic cell neoplasm. Cancer Genet 208(9):464–467.  https://doi.org/10.1016/j.cancergen.2015.04.011 CrossRefPubMedGoogle Scholar
  33. 33.
    Jardin F, Callanan M, Penther D, Ruminy P, Troussard X, Kerckaert JP, Figeac M, Parmentier F, Rainville V, Vaida I, Bertrand P, Duval AB, Picquenot JM, Chaperot L, Marolleau JP, Plumas J, Tilly H, Bastard C (2009) Recurrent genomic aberrations combined with deletions of various tumour suppressor genes may deregulate the G1/S transition in CD4+CD56+ haematodermic neoplasms and contribute to the aggressiveness of the disease. Leukemia 23(4):698–707.  https://doi.org/10.1038/leu.2008.359 CrossRefPubMedGoogle Scholar
  34. 34.
    Abdelwahab O, Levine RL (2013) Mutations in epigenetic modifiers in the pathogenesis and therapy of acute myeloid leukemia. Blood 121(18):3563–3572.  https://doi.org/10.1182/blood-2013-01-451781 CrossRefGoogle Scholar
  35. 35.
    Alayed K, Patel KP, Konoplev S, Singh RR, Routbort MJ, Reddy N, Pemmaraju N, Zhang L, Shaikh AA, Aladily TN, Jain N, Luthra R, Medeiros LJ, Khoury JD (2013) TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow. Am J Hematol 88(12):1055–1061.  https://doi.org/10.1002/ajh.23567 CrossRefPubMedGoogle Scholar
  36. 36.
    Menezes J, Acquadro F, Wiseman M, Gómez-López G, Salgado RN, Talavera-Casañas JG, Buño I, Cervera JV, Montes-Moreno S, Hernández-Rivas JM, Ayala R, Calasanz MJ, Larrayoz MJ, Brichs LF, Gonzalez-Vicent M, Pisano DG, Piris MA, Álvarez S, Cigudosa JC (2014) Exome sequencing reveals novel and recurrent mutations with clinical impact in blastic plasmacytoid dendritic cell neoplasm. Leukemia 28(4):823–829.  https://doi.org/10.1038/leu.2013.283 CrossRefPubMedGoogle Scholar
  37. 37.
    Stenzinger A, Endris V, Pfarr N, Andrulis M, Jöhrens K, Klauschen F, Siebolts U, Wolf T, Koch PS, Schulz M, Hartschuh W, Goerdt S, Lennerz JK, Wickenhauser C, Klapper W, Anagnostopoulos I, Weichert W (2014) Targeted ultra-deep sequencing reveals recurrent and mutually exclusive mutations of cancer genes in blastic plasmacytoid dendritic cell neoplasm. Oncotarget 5(15):6404–6413.  https://doi.org/10.18632/oncotarget.2223 CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Brunetti L, Di Battista V, Venanzi A, Schiavoni G, Martelli MP, Ascani S, Mecucci C, Tiacci E, Falini B (2017) Blastic plasmacytoid dendritic cell neoplasm and chronic myelomonocytic leukemia: a shared clonal origin. Leukemia 31(5):1238–1240.  https://doi.org/10.1038/leu.2017.38 CrossRefPubMedGoogle Scholar
  39. 39.
    Wang L, Yang M, Zhang X, Yang C, Huang X, Wang Z, Jin J (2017) ARID1A mutation in blastic lasmacytoid dendritic cell neoplasm. Haematologica 102(11):e470–e472.  https://doi.org/10.3324/haematol.2017.172742 CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    (2016) BET inhibitors target blastic plasmacytoid dendritic cell neoplasms. Cancer Discov 7:11.  https://doi.org/10.1158/2159-8290.CD-RW2016-226
  41. 41.
    Feuillard J, Jacob MC, Valensi F, Maynadié M, Gressin R, Chaperot L, Arnoulet C, Brignole-Baudouin F, Drénou B, Duchayne E, Falkenrodt A, Garand R, Homolle E, Husson B, Kuhlein E, Le Calvez G, Sainty D, Sotto MF, Trimoreau F, Béné MC (2002) Clinical and biologic features of CD4+ CD56+ malignancies. Blood 99(5):1556–1563.  https://doi.org/10.1182/blood.V99.5.1556 CrossRefPubMedGoogle Scholar
  42. 42.
    Reimer P, Rüdiger T, Kraemer D, Kunzmann V, Weissinger F, Zettl A, Konrad Müller-Hermelink H, Wilhelm M (2003) What is CD4+CD56+ malignancy and how should it be treated? Bone Marrow Transplant 32(7):637–646.  https://doi.org/10.1038/sj.bmt.1704215 CrossRefPubMedGoogle Scholar
  43. 43.
    Pemmaraju N, Thomas DA, Kantarjian H, O'Brien SM, Daver NG, Nazha A, Pierce S, GarciaManero G, Cortes JE, Faderl S (2012) Analysis of outcomes of patients (pts) with blastic plasmacytoid dendritic cell neoplasm (BPDCN). J Clin Oncol 30:Abstract 6578Google Scholar
  44. 44.
    Piccaluga PP, Paolini S, Sapienza MR, Pileri SA (2012) Blastic plasmacytoid dendritic cell neoplasm: is it time to redefine the standard of care? Expert Rev Hematol 5(4):353–355.  https://doi.org/10.1586/ehm.12.33 CrossRefPubMedGoogle Scholar
  45. 45.
    Aoki T, Suzuki R, Kuwatsuka Y, Kako S, Fujimoto K, Taguchi J, Kondo T, Ohata K, Ito T, Kamoda Y, Fukuda T, Ichinohe T, Takeuchi K, Izutsu K, Suzumiya J (2015) Long-term survival following autologous and allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm. Blood 125(23):3559–3562.  https://doi.org/10.1182/blood-2015-01-621268 CrossRefPubMedGoogle Scholar
  46. 46.
    Roos-Weil D, Dietrich S, Boumendil A, Polge E, Bron D, Carreras E, Iriondo Atienza A, Arcese W, Beelen DW, Cornelissen JJ, Kröger N, Milone G, Rossi G, Jardin F, Peters C, Rocha V, Sureda A, Mohty M, Dreger P, European Group for Blood and Marrow Transplantation Lymphoma, Pediatric Diseases, and Acute Leukemia Working Parties (2013) 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 121(3):440–446.  https://doi.org/10.1182/blood-2012-08-448613 CrossRefPubMedGoogle Scholar
  47. 47.
    Zhong XD, Wang LZ, Wang X, Chang J (2014) Diffuse lung metastases in a child with blastic plasmacytoid dendritic cell neoplasm and review. Eur J Pediatr 173(12):1667–1670.  https://doi.org/10.1007/s00431-013-2248-4 CrossRefPubMedGoogle Scholar
  48. 48.
    Nguyen CM, Stuart L, Skupsky H, Lee YS, Tsuchiya A, Cassarino DS (2015) Blastic Plasmacytoid dendritic cell neoplasm in the pediatric population: a case series and review of the literature. Am J Dermatopathol 37(12):924–928.  https://doi.org/10.1097/DAD.0000000000000348 CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Dietrich S, Andrulis M, Hegenbart U, Schmitt T, Bellos F, Martens UM, Meissner J, Krämer A, Ho AD, Dreger P (2011) Blastic plasmacytoid dendritic cell neoplasia (BPDC) in elderly patients: results of a treatment algorithm employing allogeneic stem cell transplantation with moderately reduced conditioning intensity. Biol Blood Marrow Transplant 17(8):1250–1254.  https://doi.org/10.1016/j.bbmt.2010.12.706 CrossRefPubMedGoogle Scholar
  50. 50.
    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. Haematologica 95(11):1873–1879.  https://doi.org/10.3324/haematol.2010.026179 CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Leitenberger JJ, Berthelot CN, Polder KD, Pro B, McLaughlin P, Jones D, Duvic M (2008) CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate. J Am Acad Dermatol 58(3):480–488.  https://doi.org/10.1016/j.jaad.2007.12.012 CrossRefPubMedGoogle Scholar
  52. 52.
    Laribi K, Denizon N, Ghnaya H, Atlassi M, Besançon A, Pineau-Vincent F, Gaulard P, Petrella T (2014) Blastic plasmacytoid dendritic cell neoplasm: the first report of two cases treated by 5-azacytidine. Eur J Haematol 93(1):81–85.  https://doi.org/10.1111/ejh.12294 CrossRefPubMedGoogle Scholar
  53. 53.
    Khwaja R, Daly A, Wong M, Mahé E, Cerquozzi S, Owen C (2016) Azacitidine in the treatment of blastic plasmacytoid dendritic cell neoplasm: a report of 3 cases. Leuk Lymphoma 57(11):2720–2722.  https://doi.org/10.3109/10428194.2016.1160084 CrossRefPubMedGoogle Scholar
  54. 54.
    Sugimoto K-J, Shimada A, Yamaguchi N, Imai H, Wakabayashi M, Sekiguchi Y, Izumi H, Ota Y, Komatsu N, Noguchi M (2013) Sustained complete remission of a limited-stage blastic plasmacytoid dendritic cell neoplasm followed by a simultaneous combination of low-dose De VIC therapy and radiation therapy: a case report and review of the literature. Int J Clin Exp Pathol 6(11):2603–2608PubMedPubMedCentralGoogle Scholar
  55. 55.
    Vassallo C, Pezzini C, Carugno A, Derlino F, Croci G, Paulli M, Borroni G (2015) Cutaneous blastic plasmacytoid dendritic cell neoplasm: successful palliative treatment with oral prednisone in an elderly patient. Br J Dermatol 172(1):298–300.  https://doi.org/10.1111/bjd.13232 CrossRefPubMedGoogle Scholar
  56. 56.
    Steinberg A, Kansal R, Wong M, Lopez A, Lim S, Lopategui J, Lill M (2011) Good clinical response in a rare aggressive hematopoietic neoplasm: plasmacytoid dendritic cell leukemia with no cutaneous lesions responding to 4 donor lymphocyte infusions following transplant. Case Rep Transplant 2011:651906–651904.  https://doi.org/10.1155/2011/651906 PubMedPubMedCentralGoogle Scholar
  57. 57.
    Unteregger M, Valentin A, Zinke-Cerwenka W, Troppan K, Deutsch A, Cerroni L, Linkesch W, Neumeister P (2013) Unrelated SCT induces long-term remission in patients with blastic plasmacytoid dendritic cell neoplasm. Bone Marrow Transplant 48(6):799–802.  https://doi.org/10.1038/bmt.2012.242 CrossRefPubMedGoogle Scholar
  58. 58.
    Angelot-Delettre F, Roggy A, Frankel AE, Lamarthee B, Seilles E, Biichle S, Royer B, Deconinck E, Rowinsky EK, Brooks C, Bardet V, Benet B, Bennani H, Benseddik Z, Debliquis A, Lusina D, Roussel M, Solly F, Ticchioni M, Saas P, Garnache-Ottou F (2015) In vivo and in vitro sensitivity of blastic plasmacytoid dentritic cell neoplasm to SL-401, an interleukin-3 receptor targeted biologic agent. Haematologica 2015:223–230.  https://doi.org/10.3324/haematol.2014.111740.CrossRefGoogle Scholar
  59. 59.
    Frankel AE, Woo JH, Ahn C, Pemmaraju N, Medeiros BC, Carraway HE, Frankfurt O, Forman SJ, Yang XA, Konopleva M, Garnache-Ottou F, Angelot-Delettre F, Brooks C, Szarek M, Rowinsky E (2014) Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood 124(3):385–392.  https://doi.org/10.1182/blood-2014-04-566737 CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Shepro D, Miller M (2014) Gemcitabine in blastic plasmacytoid dendritic cell neoplasm (CD4+ CD56+ hematodermic neoplasm). Blood 124: Abstract 5457Google Scholar
  61. 61.
    Montero J, Stephansky J, Cai T, Griffin GK, Cabal-Hierro L, Togami K, Hogdal LJ, Galinsky I, Morgan EA, Aster JC, Davids MS, LeBoeuf NR, Stone RM, Konopleva M, Pemmaraju N, Letai A, Lane AA (2017) Blastic Plasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax. Cancer Discov 7(2):156–164.  https://doi.org/10.1158/2159-8290.CD-16-0999 CrossRefPubMedGoogle Scholar
  62. 62.
    Sapienza MR, Fuligni F, Agostinelli C, Tripodo C, Righi S, Laginestra MA, Pileri A Jr, Mancini M, Rossi M, Ricci F, Gazzola A, Melle F, Mannu C, Ulbar F, Arpinati M, Paulli M, Maeda T, Gibellini D, Pagano L, Pimpinelli N, Santucci M, Cerroni L, Croce CM, Facchetti F, Piccaluga PP, Pileri SA, AIRC 5xMille consortium ‘Genetics-driven targeted management of lymphoid malignancies and the Italian Registry on Blastic Plasmacytoid Dendritic Cell Neoplasm (2014) Molecular profiling of blastic plasmacytoid dendritic cell neoplasm reveals a unique pattern and suggests selective sensitivity to NF-kB pathway inhibition. Leukemia 28(8):1606–1616.  https://doi.org/10.1038/leu.2014.64 CrossRefPubMedPubMedCentralGoogle Scholar
  63. 63.
    Agliano A, Martin-Padura I, Marighetti P, Gregato G, Calleri A, Prior C, Redrado M, Calvo A, Bertolini F (2011) Therapeutic effect of lenalidomide in a novel xenograft mouse model of human blastic NK cell lymphoma/blastic plasmacytoid dendritic cell neoplasm. Clin Cancer Res 17(19):6163–6173.  https://doi.org/10.1158/1078-0432.CCR-11-0212 CrossRefPubMedGoogle Scholar
  64. 64.
    Ulrickson ML, Puri A, Lindstrom S, Cassaday RD, De Padova N, Becker PS (2017) Gemcitabine and docetaxel as a novel treatment regimen for blastic plasmacytoid dendritic cell neoplasm. Am J Hematol 92(5):E75–E77.  https://doi.org/10.1002/ajh.24696 CrossRefPubMedGoogle Scholar
  65. 65.
    Philippe L, Ceroi A, Bôle-Richard E, Jenvrin A, Biichle S, Perrin S, Limat S, Bonnefoy F, Deconinck E, Saas P, Garnache-Ottou F, Angelot-Delettre F (2017) Bortezomib as a new therapeutic approach for blastic plasmacytoid dendritic cell neoplasm. Haematologica 102(11):1861–1868.  https://doi.org/10.3324/haematol.2017.169326 CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Suzuki K, Suzuki Y, Hama A, Muramatsu H, Nakatochi M, Gunji M, Ichikawa D, Hamada M, Taniguchi R, Kataoka S, Murakami N, Kojima D, Sekiya Y, Nishikawa E, Kawashima N, Narita A, Nishio N, Nakazawa Y, Iwafuchi H, Watanabe KI, Takahashi Y, Ito M, Kojima S, Kato S, Okuno Y (2017) Recurrent MYB rearrangement in blastic plasmacytoid dendritic cell neoplasm. Leukemia 31(7):1629–1633.  https://doi.org/10.1038/leu.2017.101 CrossRefPubMedGoogle Scholar
  67. 67.
    Kim MJ, Nasr A, Kabir B, de Nanassy J, Tang K, Menzies-Toman D, Johnston D, El Demellawy D (2017) Pediatric blastic plasmacytoid dendritic cell neoplasm: a systematic literature review. J Pediatr Hematol Oncol 39(7):528–537.  https://doi.org/10.1097/MPH.0000000000000964 CrossRefPubMedGoogle Scholar
  68. 68.
    Shi Y, Wang E (2014) Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Arch Pathol Lab Med 138(4):564–569.  https://doi.org/10.5858/arpa.2013-0101-RS CrossRefPubMedGoogle Scholar
  69. 69.
    Pintoalmeida T, Fernandes I, Sanches M, Lau C, Lima M, Alves R, Selores M (2012) A case of blastic plasmacytoid dendritic cell neoplasm. Ann Dermatol 24(2):235–237.  https://doi.org/10.5021/ad.2012.24.2.235 CrossRefGoogle Scholar
  70. 70.
    Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Miura K, Hatta Y, Sugitani M (2015) Radiation therapy for cutaneous blastic plasmacytoid dendritic cell neoplasm: a case report and review of the literature. Int J Clin Exp Med 8(5):8204–8209PubMedPubMedCentralGoogle Scholar
  71. 71.
    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 Onco 15(7):1097–1108.  https://doi.org/10.1093/annonc/mdh268 CrossRefGoogle Scholar
  72. 72.
    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.  https://doi.org/10.1038/modpathol.3800679 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Shunan Wang
    • 1
  • Xingtong Wang
    • 1
  • Mingsuo Liu
    • 1
  • Ou Bai
    • 1
  1. 1.Department of HematologyThe First Hospital of Jilin UniversityChangchunPeople’s Republic of China

Personalised recommendations