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CORRESPONDENCE
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
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_57_20

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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 May 6];66:195-6. Available from: https://www.e-ijd.org/text.asp?2021/66/2/195/313761




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

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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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
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
    
2.
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
    
3.
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
    
4.
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
    
5.
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
    


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