American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: http://www.sciepub.com/journal/ajmcr Editor-in-chief: Samy, I. McFarlane
Open Access
Journal Browser
Go
American Journal of Medical Case Reports. 2020, 8(12), 438-442
DOI: 10.12691/ajmcr-8-12-1
Open AccessCase Report

A Rare Case Report of Blastic Plasmacytoid Dendritic Cell Neoplasm

Sruthi Roopa BM1, , Ramya Ananthakrishnan1, AllwynYabesh T1, Rameez Ahamed1 and Raja Thirumalairaj1

1Department of Medical Oncology, Apollo Cancer Institute, Chennai, India

Pub. Date: August 14, 2020

Cite this paper:
Sruthi Roopa BM, Ramya Ananthakrishnan, AllwynYabesh T, Rameez Ahamed and Raja Thirumalairaj. A Rare Case Report of Blastic Plasmacytoid Dendritic Cell Neoplasm. American Journal of Medical Case Reports. 2020; 8(12):438-442. doi: 10.12691/ajmcr-8-12-1

Abstract

Blastic plasmacytoid dendritic cell neoplasm also formerly known as Blastic NK cell lymphoma is a very rare haematological malignancy. WHO defined BPDCN as a neoplasm with features of cutaneous lymphoma and/or leukaemia and designated BPDCN in a separate category under the myeloid class of neoplasms since 2008. Diagnosis of BPDCN requires both morphological evidence of plasmacytoid dendritic blast cells and immunohistochemical positivity for CD123, CD4, CD56 and TLC-1. Unlike other haematological malignancies there are no established optimal chemotherapy regimens for BPCDN although standard chemotherapeutic regimens used for induction treatment of AML, ALL and high grade lymphoma have shown complete remission rates albeit for a short duration, with ALL regimens having a higher remission percentage among both adults and children. Here we present a case of BPDCN without skin involvement.

Keywords:
BPCDN pathological features Immunohistochemical criteria prognosis chemotherapy role

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Figures

Figure of 5

References:

[1]  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.
 
[2]  Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016; 127(20): 2391-2405.
 
[3]  Jacob MC, Chaperot L, Mossuz P, et al. CD4+ CD56+ lineage negative malignancies: a new entity developed from malignant early plasmacytoid dendritic cells. Haematologica. 2003; 88(8): 941-955.
 
[4]  Feuillard J, Jacob MC, Valensi F, et al. Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood. 2002; 99(5): 1556-1563.
 
[5]  Pagano L, Valentini CG, Pulsoni A, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013; 98(2): 239-246.
 
[6]  Brunetti L, Di Battista V, Venanzi A, et al. Blastic plasmacytoid dendritic cell neoplasm and chronic myelomonocytic leukemia: a shared clonal origin. Leukemia. 2017; 31(5): 1238-1240.
 
[7]  Foong HB, Chong M, Taylor EM, Carlson JA, Petrella T. Blastic plasmacytoid dendritic cell neoplasm in an elderly woman. Med J Malaysia. 2013; 68(2): 161-163.
 
[8]  Tsagarakis NJ, Kentrou NA, Papadimitriou KA, et al. Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the Hellenic Dendritic Cell Leukemia Study Group. Leuk Res. 2010; 34(4): 438-446.
 
[9]  Wang H, Cao J, Hong X. Blastic plasmacytoid dendritic cell neoplasm without cutaneous lesion at presentation: case report and literature review. Acta Haematol. 2012; 127(2): 124-127.
 
[10]  Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016; 2016(1): 16-23.
 
[11]  Deotare U, Yee KW, Le LW, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-Color flow cytometry diagnosis and HyperCVAD therapy. Am J Hematol. 2016; 91(3): 283-286.
 
[12]  Laribi K, Denizon N, Besançon A, et al. Blastic Plasmacytoid Dendritic Cell Neoplasm: From Origin of the Cell to Targeted Therapies. Biol Blood Marrow Transplant. 2016; 22(8): 1357-1367.
 
[13]  Lin C, Wu M, Kuo T, Lu P. Cutaneous blastic plasmacytoid dendritic cell neoplasm: Report of a case and review of the literature. Dermatologica Sinica. 2017; 35(2): 96-99.
 
[14]  Riaz W, Zhang L, Horna P, Sokol L. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control.2014; 21(4): 279-289.
 
[15]  Petrella T, Dalac S, Maynadié M, et al. CD4+ CD56+ cutaneous neoplasms: a distinct hematological entity? Groupe Français d'Etude des Lymphomes Cutanés (GFELC). Am J Surg Pathol. 1999; 23(2): 137-146.
 
[16]  Leroux D, Mugneret F, Callanan M, et al. 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. 2002; 99(11): 4154-4159.
 
[17]  Sakamoto K, Katayama R, Asaka R, et al. Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response. Leukemia. 2018; 32(12): 2590-2603.
 
[18]  Sumarriva Lezama L, Chisholm KM, Carneal E, et al. An analysis of blastic plasmacytoid dendritic cell neoplasm with translocations involving the MYC locus identifies t(6;8)(p21;q24) as a recurrent cytogenetic abnormality. Histopathology. 2018; 73(5): 767-776.
 
[19]  Boddu PC, Wang SA, Pemmaraju N, Tang Z, Hu S, Li S, Xu J, Medeiros LJ, Tang G. 8q24/MYC rearrangement is a recurrent cytogenetic abnormality in blastic plasmacytoid dendritic cell neoplasms. Leuk. Res 2018, 66, 72-78.
 
[20]  Swerdlow SH, Camo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. World health organization classification of tumours. 4th ed. International Agency for research on cancer, Lyon, France 2017.
 
[21]  Cota C, Vale E, Viana I, et al. Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol. 2010; 34(1): 75-87.
 
[22]  Pemmaraju N, Sweet K, Lane A et al. Results of pivotal phase 2 trial of SL 401 patients with blastic plasmacytoid dendritic cell neoplasm. EHA learn cent. 2018; 214438
 
[23]  Pemmaraju N, Lane AA, Sweet KL, et al. Tagraxofusp in blastic plasmacytoid dendritic-cell neoplasm. N Engl J Med. 2019; 380(17): 1628-1637
 
[24]  Montero J, Stephansky J, Cai T, et al. Blastic Plasmacytoid Dendritic Cell Neoplasm Is Dependent on BCL2 and Sensitive to Venetoclax. Cancer Discov. 2017; 7(2): 156-164.
 
[25]  Philippe L, Ceroi A, Bôle-Richard E, et al. Bortezomib as a new therapeutic approach for blastic plasmacytoid dendritic cell neoplasm. Haematologica. 2017; 102(11): 1861-1868.
 
[26]  Ceribelli M, Hou ZE, Kelly PN, et al. A Druggable TCF4- and BRD4-Dependent Transcriptional Network Sustains Malignancy in Blastic Plasmacytoid Dendritic Cell Neoplasm. Cancer Cell. 2016; 30(5): 764-778.
 
[27]  Emadali A, Hoghoughi N, Duley S, et al. Haploinsufficiency for NR3C1, the gene encoding the glucocorticoid receptor, in blastic plasmacytoid dendritic cell neoplasms. Blood. 2016; 127(24): 3040-3053.