Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
Users online: 1210  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page

Table of Contents 
Year : 2021  |  Volume : 66  |  Issue : 2  |  Page : 195-196
Novel ultrastructural findings of blastic plasmacytoid dendritic cell neoplasm

Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Tottori University Faculty of Medicine, Yonago, Japan

Date of Web Publication16-Apr-2021

Correspondence Address:
Ayano Ikeda
Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Tottori University Faculty of Medicine, Yonago
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_57_20

Rights and Permissions

How to cite this article:
Sugita K, Ikeda A, Kimura R, Yamamoto O. Novel ultrastructural findings of blastic plasmacytoid dendritic cell neoplasm. Indian J Dermatol 2021;66:195-6

How to cite this URL:
Sugita K, Ikeda A, Kimura R, Yamamoto O. Novel ultrastructural findings of blastic plasmacytoid dendritic cell neoplasm. Indian J Dermatol [serial online] 2021 [cited 2021 Oct 16];66:195-6. Available from:

A 64-year-old man presented with a 2-year history of cutaneous lesions on his face, trunk, left arm, and right leg. Physical examination revealed erythematous patches on his forehead, right ear, cheeks, lower jaw, right forearm, left lower thigh, and trunk [Figure 1]a. Superficial lymph nodes were not palpable. He was otherwise healthy and was taking no medication. Peripheral blood showed white blood cells of 1.6 × 103/μL, red blood cells of 3.46 × 106/μL, hemoglobin of 11.0 g/dL, and platelet count of 19 × 103/μL. The differential count showed 62% lymphocytes, 34% neutrophils, and 4% monocytes without atypical blood cells. Blood chemistry values were within normal ranges. A skin biopsy from the lower jaw revealed a diffuse sheet of small to medium-sized tumor cells with poor cytoplasm throughout the dermis and with the minimal involvement of the overlying epidermis [Figure 1]b. These cells were positive for CD4 [Figure 1]c, CD56 [Figure 1]d, CD123, and terminal deoxynucleotidyl transferase (TdT) but negative for cytoplasmic CD3, CD8, CD20, CD34, CD1a, and c-kit [Figure 1]e. Monoclonal T-cell receptor and immunoglobulin gene rearrangements were not detected. Flow cytometric analysis of the skin lesion revealed that CD4+and CD123+tumor cells accounted for 32.9% of the CD45-positive cells in the skin. However, the majority of the cells were negative for CD303 [Figure 1]f. A mild increase in mast cells presenting as evenly distributed scattered cells was seen among tumor cells in toluidine blue-stained sections. Transmission electron microscopy (TEM) showed that the tumor cells had microvillous processes on the cell surface. The nucleus had a prominent nucleolus and a moderate amount of heterochromatin. Intriguingly, the tumor cells were composed of mature dendritic cells [Figure 1]g. There were many rough endoplasmic reticulum and transport vesicles in the mature dendritic cell neoplasm. Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) demonstrated multiple foci of increased uptake in the skin, spleen, lymph nodes, and bone marrow. Based on these findings, the skin lesion was diagnosed as blastic plasmacytoid dendritic cell neoplasm (BPDCN) associated with multiple organ involvement. The patient was treated with 2 cycles of hyper CVAD/MA chemotherapy (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, and cytarabine). The treatment resulted in partial response, and his skin lesions markedly improved over the next month. Thereafter, the patient was followed-up for 16 months without the recurrence.
Figure 1: (a) There was invasive erythematous plaque in the right ear and right cheek. (b) A skin biopsy from the lower jaw revealed diffuse sheets of small cytoplasmic cells with poor cytoplasm throughout the dermis and with minimal involvement of the overlying epidermis (H&E, 10 ×). (c) Atypical cells expressed CD4 (40 ×). (d) Atypical cells expressed CD56 (40 ×). (e) c-kit-negative atypical cells (40 ×) (f) Flow cytometry analysis of the tumor. (g) Transmission electron microscopy (TEM) showed mature plasmacytoid dendritic cells

Click here to view

BPDCN is a rare aggressive lymphoma initially identified as the leukemic counterpart of plasmacytoid dendritic cells.[1],[2] There is no description of skin BPDCN with regard to TEM findings and maturity status. It has been reported that BPDCN can be categorized into three maturational stages by CD34 and c-kit stainings.[3] Based on our findings, our case is a c-kit and CD34-double-negative mature immunophenotype. In previous studies in which TEM of bone marrow BPDCN was performed, CD34 and c-kit immunostainings were not performed, and thus the maturation stage was not clarified. [4,5] Although those cases showed a rough endoplasmic reticulum, our case also showed transport vesicles, which support the mature phenotype of BPDCN. In conclusion, the combination of immunohistochemical and ultrastructural analyses enables a more accurate diagnosis of this clinically heterogeneous tumor to be made.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Jacob MC, Chaperot L, Mossuz P, Feuillard J, Valensi F, Leroux D, et al. CD4+CD56+lineage negative malignancies: Anew entity developed from malignant early plasmacytoid dendritic cells. Haematologica 2003;88:941-55.  Back to cited text no. 1
Chaperot L, Bendriss N, Manches O, Gressin R, Maynadie M, Trimoreau F, et al. Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood 2001;97:3210-7.  Back to cited text no. 2
Martin-Martin L, Lopez A, Vidriales B, Caballero MD, Rodrigues AS, Ferreira SI, et al. Classification and clinical behavior of blastic plasmacytoid dendritic neoplasms according to their maturation-associated immunophenotypic profile.Oncotarget 2015;22:19204-16.  Back to cited text no. 3
Takiuchi T, Maruoka H, AokiK, Kato A, Ono Y, Nagano S, et al. Leukemic maifestation of blastic plasmacytoid dendritic cell neoplasm lacking skin lesion: A borderline case between acute monocytic leukemia. J Clin Exp Hematopathol2012;52:107-11.  Back to cited text no. 4
Ru Y, Zhang P, Dong S, Wang H, Zhao S, Mi Y, et al. Morphologic characteristics of blastic plasmacytoid dendritic cell neoplasm: A case report. Ultrastruct Pathol2014;38:66-8.  Back to cited text no. 5


  [Figure 1]


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,196 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded32    
    Comments [Add]    

Recommend this journal