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Table of Contents 
CASE REPORT
Year : 2020  |  Volume : 65  |  Issue : 3  |  Page : 217-221
Blastic plasmacytoid dendritic cell neoplasm: Case report and literature overview


1 Center of Postgraduate Medical Education, Novosibirsk National Research State University, Novosibirsk, Russian Federation
2 Center of Postgraduate Medical Education, Novosibirsk National Research State University; Novosibirsk Research Institute of Fundamental and Clinical Immunology, Novosibirsk, Russian Federation
3 Novosibirsk Research Institute of Fundamental and Clinical Immunology, Novosibirsk, Russian Federation
4 Novosibirsk Scientific Research Institute of Tuberculosis, Novosibirsk, Russian Federation

Date of Web Publication14-Apr-2020

Correspondence Address:
Anastasiia Kolerova
Center of Postgraduate Medical Education, Novosibirsk National Research State University, Pirogova, 630090 Novosibirsk
Russian Federation
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_235_18

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   Abstract 


Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a malignancy with high frequency of skin involvement. A 39-year-old Caucasian female was suffering from weakness, myalgia, and skin eruption, which appeared during treatment of chlamydiosis with antibiotics in July 2016. Based on clinical presentation, laboratory investigations, and histological examination of skin and bone marrow biopsy, a diagnosis of BPDCN with the involvement of skin, bone marrow, and central nervous system was made. The patient was put on acute lymphoblastic leukemia-like chemotherapy and achieved complete remission in November 2016, the eruption regressed. In January 2017, allogeneic bone marrow transplantation from matched sibling was performed. Since May 2017, the cutaneous relapse with loss of CD56 expression has developed. This clinical case demonstrates the importance of laboratory tests. Histological examination helps to clarify a diagnosis of cutaneous lymphoma; however, a specific type of lymphoma needs immunohistochemical analysis. In our case, BPDCN at the initial stage presented like a systemic vasculitis.


Keywords: Blastic plasmacytoid dendritic cell neoplasm, cutaneous lymphoma, cutaneous oncology


How to cite this article:
Kolerova A, Sergeeva I, Krinitsyna J, Pronkina N, Sizikova S, Filimonov P, Kryuchkova I. Blastic plasmacytoid dendritic cell neoplasm: Case report and literature overview. Indian J Dermatol 2020;65:217-21

How to cite this URL:
Kolerova A, Sergeeva I, Krinitsyna J, Pronkina N, Sizikova S, Filimonov P, Kryuchkova I. Blastic plasmacytoid dendritic cell neoplasm: Case report and literature overview. Indian J Dermatol [serial online] 2020 [cited 2020 May 30];65:217-21. Available from: http://www.e-ijd.org/text.asp?2020/65/3/217/282445





   Introduction Top


Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a malignancy with high frequency of skin involvement.[1] It develops from the plasmacytoid dendritic precursors of the myeloid line. Dendrocytes (DCs) are divided into two subtypes: classical CD11c+/CD123− cells and CD123+/CD11c−. Cells from the first subtype are presenter cells whose main function is triggering the primary immune response through the activation of naive T-lymphocytes via IL-12. DCs of the second type play an important role in antiviral defense through production of interferon-1. Plasmacytoid dendritic cells are absent in normal skin but migrate to the skin during wound healing, infections, inflammation, and neoplasms.[2]

The first published case of BPDCN was described by Adachi et al. in 1994. Previously known as NK-cell blastic lymphoma, it is three times more prevalent in men than in women and occurs primarily in patients older than 60 years, even though the disease can manifest at any age.[3] Prognosis is poor; the average survival rate is 14 months, with a 2-year survivability of 33% among patients, and a 5-year survival rate of 6%.[1]


   Case Report Top


A 39-year-old Caucasian female was suffering from weakness, malaise, numbness of the distal parts of the limbs, weakness in the hands, myalgia, and skin eruption. At the time of examination (September, 2016), the eruption was presented with multiple sharply marginated bluish dense papules, prominent over the skin surface. The patient had edema of the shins. During regular medical examination in May 2016, leukopenia of 1.9 × 109/l and hemoglobin 98 g/l were observed. The rash first appeared during treatment of chlamydiosis with antibiotics in July 2016. The eruption presented with scarlet papules on the skin of shins and forearms. Systemic vasculitis was diagnosed; lymphoma was not included in differential diagnosis. The patient was treated with methylprednisolone 25 mg and prednisolone 25 mg per day with no effect. In September, condition of the patient worsened, and the eruption became widespread [Figure 1] and [Figure 2]. In the analysis of blood, pancytopenia increased, so then a bone marrow examination and a biopsy of skin were performed. The bone marrow aspirate showed infiltration of atypical lymphoid cells of medium and large sizes with a rounded nucleus, variable basophilic cytoplasm, reduced hematopoietic cells including loss of megakaryocytes. The immunohistochemical examination showed positive expression of CD4, CD56. Skin biopsy showed a massive monomorphic infiltration of medium-sized cells with moderately polymorphic dispersed chromatin, a narrow cytoplasm all over the dermis and subcutis. Nuclei were clear, and a significant amount of mitosis was observed. There were no signs of epidermotropism [Figure 3]. Cells were characterized by positive immunohistochemical reactions for CD4, CD56, S100, Ki67 (70%) [Figure 4]. Cytology analysis of cerebrospinal fluid showed similar tumor cells. Based on these findings, a diagnosis of BPDCN with involvement of skin, bone marrow, and central nervous system was made. The patient was started on acute lymphoblastic leukemia-like chemotherapy (I induction phase: dexamethasone, daunorubicin, vinblastine, L-asparaginase; II induction phase: purinethol, endoxan, cytosar, L-asparaginase), with intrathecal administration of methotrexate, cytarabine, and dexamethasone.
Figure 1: Cutaneous changes on the shins

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Figure 2: Cyanotic-pink dense papules and plaques at the initial presentation

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Figure 3: Skin biopsy found a massive monomorphic infiltration of medium-sized cells with no signs of epidermotropism (H and E,×40 and ×100)

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Figure 4: Immunohistochemical staining of skin biopsy showing diffusely positive CD4, CD56, S100, Ki-67 cells with proliferative activity 70% (×400)

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The patient achieved complete remission in bone marrow and central nervous system when tested in November 2016, and the eruption regressed completely. In January 2017, allogeneic bone marrow transplantation from matched sibling was performed. Since May 2017, the relapse of only the skin lesions had developed. Immunohistochemical examination showed a loss of CD56 expression, which was unusual.


   Discussion Top


BPDCN is an extremely rare disease; therefore, the clinical features are described primarily in only a few published clinical cases. A literature review made it possible to reveal certain characteristics of this pathology.

The disease manifests as an eruption in 90% of all cases. Typically, the eruption is widespread, presents in the form of papules, plaques, nodules with clear demarcation of brownish-red, purple, or cyanotic color. The rash is often itchy, painful, but may be asymptomatic. The size of these elements varies from a few millimeters to 3–4 cm. In rare cases, nodules and plaques with a diameter of up to 10 cm have been observed. The disease may also present as erythema, hyperpigmentation, purpura, or ulcers with necrosis. Mucous membranes are rarely involved and only observed in 10% of patients.[2] The described clinical case clearly demonstrates the characteristics of BPDCN. The disease manifested with leukopenia and anemia. A skin lesion appeared after 2 months characterized by scarlet papules on the legs and forearms. Within 4 months, rash had become widespread. The eruption finally presented with multiple sharply marginated bluish dense papules, prominent over the skin surface [Table 1].
Table 1: Published cases of blastic plasmacytoid dendritic cell neoplasm

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Lymphadenopathy and splenomegaly are observed in 40%–50% and 20% of patients, respectively. Pathological changes in the peripheral blood are found in 60%–90%. Fulminant leukemia typically occurs in the terminal stage of the disease.[3] The central nervous system is involved in the pathological process in isolated cases, one of which is the present case.

The histological picture of BPDCN is characterized by diffuse dermal infiltrate confined around the vessels and appendages of the skin, especially during the early stages. Epidermotropism is rarely observed. In cases where the size of the tumor is large, subcutaneous tissues may be involved.[4],[5] Main markers of the disease are CD4, CD56, CD123 without specific markers of B-cells and myelomonocytic cells. Expression of CD4 and CD56 may be weak sometimes. Cells of CD4/CD56 hemoderma are characterized by the presence of CD43, HLA-DR, and CD45RA, which are expressed on membranes of B-cells and naive T-lymphocytes.[4],[6] CD123, IL-3 alpha-chain receptor, is a specific marker of BPDCN; however, it is presented on cells in myelomonocytic leukemia. Blood dendritic cell antigen-2 is also a particular marker. T-cell leukemic marker-1 is a regulator of Akt-kinase, lymphoid protooncogene. It is presented on membranes of pDCs and B-cells, and it is absent on mature DC, hematopoietic cells, and T-cells. This marker is typical for early stages of the acute T-cell lymphoblastic leukemia and T-cell prolymphocytic leukemia and finally, for BPDCN.[3]

Cutaneous extramedullary myeloid sarcoma and myelomonocytic leukemia usually mimic BPDCN. There are specific markers to differentiate these pathologies[6] [Table 2].
Table 2: Differential diagnosis between blastic plasmacytoid dendritic cell neoplasm and cutaneous extramedullary myeloid sarcoma, myelomonocytic leukemia

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Extranodal NK/T-cell lymphoma should be excluded before making the diagnosis of BPDCN. This disease is associated with severe course and poor prognosis. Histological findings include angiocentric foci of growth with necrosis. Immunohistochemical phenotype comprises of CD56+, CD3+, TLA1+, granzyme+. Sometimes typical T-cell lymphomas can represent CD56 on the surface of tumor cells. However, T cell receptor (TCR) rearrangement is detected often, and other T-cell markers.[13]


   Conclusions Top


This clinical case demonstrates the importance of laboratory tests. The first manifestations of the cutaneous lymphoma in our patient were anemia and leukopenia, which can be detected using a routine blood test. When detecting such changes, it is necessary to exclude malignancies and lymphoproliferative disorders. Histological examination helps to clarify a diagnosis of cutaneous lymphoma; however, a specific type of lymphoma needs immunohistochemical analysis.

Cutaneous lymphomas can mimic benign skin dermatoses. In our case, BPDCN mimicked a systemic vasculitis. Only because of the disease progression despite treatment the diagnosis of lymphoma was made. Therefore, it is important to know this entity to be able to recognize it at the early stages.

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.[14]



 
   References Top

1.
Saadeh D, Kurban M, Abbas O. Plasmacytoid dendritic cell role in cutaneous malignancies. J Dermatol Sci 2016;83:3-9.  Back to cited text no. 1
    
2.
Dantas FE, de Almeida Vieira CA, de Castro CC, Neto GC, Matos DM. Blastic plasmacytoid dendritic cell neoplasm without cutaneous involvement: A rare disease with a rare presentation. Acta Oncol 2012;51:139-41.  Back to cited text no. 2
    
3.
Zhang YW, Zhong JH, Chen XL, Xiao F, Chen FY. Blastic plasmacytoid dendritic cell neoplasm: A case report and literature review. Exp Ther Med 2016;12:319-22.  Back to cited text no. 3
    
4.
Chou YH, Lin RY, Lee MS, Wu HP, Lin CP. Bruise-like cutaneous lesions as the early presentation of blastic plasmacytoid dendritic cell neoplasm. Dermatol Sin 2014;24:101-6.  Back to cited text no. 4
    
5.
Kacar Döger F, Dikicioǧlu Çetin E, Hekimgil M, Özdoǧan Uslu M, Kadıköylü G, Özsan N, et al. Plasmacytoid dendritic cell tumor: A case report. Turk J Haematol 2011;28:312-6.  Back to cited text no. 5
    
6.
Li Y, Li Z, Lin HL, Chen XH, Li B. Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: Case report and review of the literature. Pathol Res Pract 2011;207:55-9.  Back to cited text no. 6
    
7.
Gurgen J, Hogan D, Muller K, Flowers J, Sexton W. CD4+/CD56+ tdT+ haematodermic neoplasm (previously called blastic natural killer cell lymphoma) in a patient with chronic human T-cell leukaemia virus type 1 infection: A previously unreported association. Br J Dermatol 2010;162:1395-7.  Back to cited text no. 7
    
8.
Eros N, Marschalkó M, Balassa K, Hídvégi B, Szakonyi J, Ilniczky S, et al. Central nervous system involvement in CD4+/CD56+ hematodermic neoplasm: A report of two cases. J Neurooncol 2010;97:301-4.  Back to cited text no. 8
    
9.
Fu Y, Fesler M, Mahmud G, Bernreuter K, Jia D, Batanian JR, et al. Narrowing down the common deleted region of 5q to 6.0 MB in blastic plasmacytoid dendritic cell neoplasms. Cancer Genet 2013;206:293-8.  Back to cited text no. 9
    
10.
Rakheja D, Fuda F, Vandergriff T, Boriack R, Medeiros BC, Frankel AE, et al. Increased plasma d-2-hydroxyglutarate in isocitrate dehydrogenase 2-mutated blastic plasmacytoid dendritic cell neoplasm. Hum Pathol 2015;46:322-6.  Back to cited text no. 10
    
11.
Rauh MJ, Rahman F, Good D, Silverman J, Brennan MK, Dimov N, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Leuk Res 2012;36:81-6.  Back to cited text no. 11
    
12.
Lin CY, Wu MY, Kuo T, Lu PH. Cutaneous blastic plasmacytoid dendritic cell neoplasm: Report of a case and review of the literature. Dermatol Sin 2017;35:96-9.  Back to cited text no. 12
    
13.
Huang YY, Liu YR, Li K, Li K, Liu SH. A woman with rare blastic plasmacytoid dendritic cell neoplasm on the face. Oral Surg Oral Med Oral Pathol Oral Radiol 2016;121:e16-8.  Back to cited text no. 13
    
14.
Toya T, Nishimoto N, Koya J, Nakagawa M, Nakamura F, Kandabashi K, et al. The first case of blastic plasmacytoid dendritic cell neoplasm with MLL-ENL rearrangement. Leuk Res 2012;36:117-8.  Back to cited text no. 14
    


    Figures

  [Figure 2], [Figure 1], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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