CHEN Xiaojun, LIU Yanquan, HU Xiaomei, HUANG Surong, SHEN Jianzhen. Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report[J]. Medical Journal of Peking Union Medical College Hospital, 2022, 13(1): 124-129. DOI: 10.12290/xhyxzz.2021-0465
Citation: CHEN Xiaojun, LIU Yanquan, HU Xiaomei, HUANG Surong, SHEN Jianzhen. Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report[J]. Medical Journal of Peking Union Medical College Hospital, 2022, 13(1): 124-129. DOI: 10.12290/xhyxzz.2021-0465

Regimen of Azacitidine Combined with GemOx in the Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Funds: 

National Natural Science Foundation of China 81300428

Fujian Blood Medical Center Construction Project Fujian Mdical Administration[2017] No. 171

National and Fujian Provincial Key Clinical Specialty Construction Project Fujian Mdical Letter[2011] No. 1018

National and Fujian Provincial Key Clinical Specialty Construction Project Fujian Health Science and Education[2012] No. 149

More Information
  • Corresponding author:

    SHEN Jianzhen, E-mail: doctorsjz@163.com

  • Received Date: June 11, 2021
  • Accepted Date: July 28, 2021
  • Available Online: September 14, 2021
  • Issue Publish Date: January 29, 2022
  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly malignant hematological tumor derived from the precursor cells of plasmacytoid dendritic cells. BPDCN that primarily originates in the nasal cavity is clinically rare and easily misdiagnosed or missed. At present, there is no standard treatment for BPDCN in China and abroad. This article reported a female patient with BPDCN primarily in the nasal cavity. The regimen of Azacitidine combined with GemOx had a good clinical effect on this patient and significantly controlled the condition and improved the clinical symptoms. This article analyzed and discussed the clinical diagnosis and treatment of this patient, aiming to improve clinicians' understanding of rare BPDCN.
  • [1]
    Sweet K. Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment[J]. Curr Opin Hematol, 2020, 27: 103-107. DOI: 10.1097/MOH.0000000000000569
    [2]
    Venugopal S, Zhou S, El Jamal SM, et al. Blastic plasmacytoid dendritic cell neoplasm-current insights[J]. Clin Lymphoma Myeloma Leuk, 2019, 19: 545-554. DOI: 10.1016/j.clml.2019.06.002
    [3]
    Deconinck E, Petrella T, Garnache Ottou F. Blastic plasmacytoid dendritic cell neoplasm: clinical presentation and diagnosis[J]. Hematol Oncol Clin North Am, 2020, 34: 491-500. DOI: 10.1016/j.hoc.2020.01.010
    [4]
    Kim MJ, Nasr A, Kabir B, et al. Pediatric blastic plasmacytoid dendritic cell neoplasm: a systematic literature review[J]. J Pediatr Hematol Oncol, 2017, 39: 528-537. DOI: 10.1097/MPH.0000000000000964
    [5]
    Li Y, Sun V, Sun W, et al. Blastic plasmacytoid dendritic cell neoplasm in children[J]. Hematol Oncol Clin North Am, 2020, 34: 601-612. DOI: 10.1016/j.hoc.2020.01.008
    [6]
    Garnache-Ottou F, Vidal C, Biichlé S, et al. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients[J]. Blood Adv, 2019, 3: 4238-4251. DOI: 10.1182/bloodadvances.2019000647
    [7]
    Sakamoto K, Takeuchi K. Cytogenetics of blastic plasmacytoid dendritic cell neoplasm: chromosomal rearrangements and DNA copy-number alterations[J]. Hematol Oncol Clin North Am, 2020, 34: 523-538. DOI: 10.1016/j.hoc.2020.01.003
    [8]
    Pagano L, Valentini CG, Grammatico S, et al. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches[J]. Br J Haematol, 2016, 174: 188-202. DOI: 10.1111/bjh.14146
    [9]
    Kerr D 2nd, Zhang L, Sokol L. Blastic Plasmacytoid Dendritic Cell Neoplasm[J]. Curr Treat Options Oncol, 2019, 20: 9. DOI: 10.1007/s11864-019-0605-x
    [10]
    Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm[J]. Hematology Am Soc Hematol Educ Program, 2016, 2016: 16-23. DOI: 10.1182/asheducation-2016.1.16
    [11]
    Hirner JP, O'Malley JT, LeBoeuf NR. Blastic Plasmacytoid Dendritic Ceu Neoplasm: the Dermatologist's Perspective[J]. Hematol Oncol Clin North Am, 2020, 34: 501-509. DOI: 10.1016/j.hoc.2020.01.001
    [12]
    Rai MP, Bedi PS, Kandola SK, et al. Blastic plasmacytoid dendritic cell neoplasm[J]. Clin Case Rep, 2018, 6: 770-772. DOI: 10.1002/ccr3.1457
    [13]
    Liu YQ, Shen JZ, Awal IM, et al. CD56-positive diffuse large B-cell lymphoma/leukemia with BCL6/MYC double-hit and multiple gene mutations: an indicator of poor prognosis[J]. J Int Med Res, 2020, 48: 300060520918087.
    [14]
    Sobas M, Montesinos P, Boluda B, et al. An analysis of the impact of CD56 expression in de novo acute promyelocytic leukemia patients treated with upfront all-trans retinoic acid and anthracycline-based regimens[J]. Leuk Lymphoma, 2019, 60: 1030-1035. DOI: 10.1080/10428194.2018.1516875
    [15]
    Rai S, Singh S, Gupta R. Prognostic significance of CD56 and CD7 in acute myeloid leukaemia and their outcome[J]. Am J Blood Res, 2020, 10: 109-117.
    [16]
    Sapienza MR, Pileri S. Molecular Features of Blastic Plasmacytoid Dendritic Cell Neoplasm: DNA Mutations and Epigenetics[J]. Hematol Oncol Clin North Am, 2020, 34: 511-521. DOI: 10.1016/j.hoc.2020.01.002
    [17]
    Bohl SR, Bullinger L, Rücker FG. Epigenetic therapy: azacytidine and decitabine in acute myeloid leukemia[J]. Expert Rev Hematol, 2018, 11: 361-371. DOI: 10.1080/17474086.2018.1453802
    [18]
    Pemmaraju N, Lane AA, Sweet KL, et al. Tagraxofusp in Blastic Plasmacytoid Dendritic-Cell Neoplasm[J]. N Engl J Med, 2019, 380: 1628-1637. DOI: 10.1056/NEJMoa1815105
    [19]
    Khwaja R, Daly A, Wong M, et al. Azacitidine in the treatment of blastic plasmacytoid dendritic cell neoplasm: a report of 3 cases[J]. Leuk Lymphoma, 2016, 57: 2720-2722. DOI: 10.3109/10428194.2016.1160084
    [20]
    Montero J, Stephansky J, Cai T, et al. Blastic Plasmacytoid Dendritic Cell Neoplasm Is Dependent on BCL2 and Sensitive to Venetoclax[J]. Cancer Discov, 2017, 7: 156-164. DOI: 10.1158/2159-8290.CD-16-0999
    [21]
    Bôle-Richard E, Fredon M, Biichlé S, et al. CD28/4-1BB CD123 CAR T cells in blastic plasmacytoid dendritic cell neoplasm[J]. Leukemia, 2020, 34: 3228-3241. DOI: 10.1038/s41375-020-0777-1
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