Blastic plasmacytoid dendritic cell neoplasm
a case report from a hospital in Espírito Santo, Brazil
DOI:
https://doi.org/10.47456/rbps.v27i1.49729Keywords:
Hematologic neoplasms, Dendritic cells, Rare diseases, Differential diagnosis, Palliative careAbstract
Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with a poor prognosis, slight male predominance, and cutaneous and systemic manifestations. Objective: To describe the clinical, diagnostic, and therapeutic course of a patient with blastic plasmacytoid dendritic cell neoplasm and to discuss the challenges associated with disease recognition and management in light of the literature. Methods: This descriptive, retrospective clinical case report was based on the analysis of clinical, laboratory, imaging, histopathological, and therapeutic data recorded in the patient’s medical records. The case was discussed in the context of a narrative review of the literature. Case report: A 52-year-old woman presented with weight loss, fever, and skin lesions and subsequently developed anemia, leukocytosis, thrombocytopenia, splenomegaly, lymphadenopathy, cutaneous involvement, and refractoriness to chemotherapy. The diagnosis was confirmed by immunohistochemical analysis of an axillary lymph node. The patient received different lines of treatment without a significant response and was subsequently referred to palliative care. Conclusion: BPDCN poses major diagnostic and therapeutic challenges. This report illustrates the clinical course and treatment difficulties associated with a still poorly recognized entity and emphasizes the importance of early clinical suspicion and referral for specialized investigation.
Downloads
References
Almeida YCF, Sampaio GP, Campos CCAP, Mendes PM, Dourado KMC. Neoplasia de células blásticas dendríticas plasmocitoides: relato de caso. Hematol Transfus Cell Ther. 2021;43(Suppl 1):S168-S169.
Khoury JD. Blastic plasmacytoid dendritic cell neoplasm. Curr Hematol Malig Rep. 2018;13(6):477-483.
Pagano L, Valentini CG, Grammatico S, Pulsoni A. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
Wang X, Gniadecki R. Clinical characteristics and outcomes of blastic plasmacytoid dendritic cell neoplasm: a population-based study. J Hematol. 2020;9(2):35-43.
Azambuja AP, Gevert F, Merhy R, Queiroz M, Nunes EC. Uso do TCL1 como auxílio no diagnóstico diferencial das leucemias de células dendríticas plasmocitoides. Hematol Transfus Cell Ther. 2023;45(Suppl 1):S6.
Jain A, Sweet K. Blastic plasmacytoid dendritic cell neoplasm. J Natl Compr Canc Netw. 2023;21(5):515-521.
Pinto KL, Accioly Filho JW, Jorge IF, Avelino ALN, Mota TCT, Dantas AMM, et al. Neoplasia de células blásticas dendríticas plasmocitoides com manifestação cutânea exuberante: relato de caso. Rev Med UFC. 2017;57(3):58-61.
Laribi K, Denizon N, Besançon A, Farhi J, Lemaire P, Sandrini J, et al. Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biol Blood Marrow Transplant. 2016;22(8):1357-1367.
Nomburg J, Bullman S, Chung SS, Togami K, Walker MA, Griffin GK, et al. Comprehensive metagenomic analysis of blastic plasmacytoid dendritic cell neoplasm. Blood Adv. 2020;4(6):1006-1011.
Micolich V, Peña C, Figueroa F, Briones V, Vidal C, Chandia M. Características clínicas de neoplasia de células dendríticas plasmocitoides blásticas en Chile: reporte de 10 casos. Rev Med Chil. 2023;151(9):1201-1206.
Andrade MLL, Amorim L, Santiago M, Lemos L. Neoplasia de células blásticas dendríticas plasmocitoides tratada com venetoclax e azacitidina: relato de caso. Hematol Transfus Cell Ther. 2020;42(Suppl 2):S169-S170.
Lencastre A, Cabete J, João A, Farinha P, Ferreira G, Lestre S. Blastic plasmacytoid dendritic cell neoplasm. An Bras Dermatol. 2013;88(6 Suppl 1):158-161.
Zhang Y, Wang W, Li Y, Wang Z. Blastic plasmacytoid dendritic cell neoplasm without cutaneous involvement: a case report. Exp Ther Med. 2016;12(1):319-322.
Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246.
Garnache-Ottou F, Feuillard J, Ferrand C, Biichle S, Trimoreau F, Seilles E, et al. Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol. 2009;145(5):624-636.
Pemmaraju N, Konopleva M. Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm. Blood Adv. 2020;4(16):4020-4027.
Yun S, Chan O, Kerr D, Vincelette ND, Idrees A, Mo Q, et al. Survival outcomes in blastic plasmacytoid dendritic cell neoplasm by first-line treatment and stem cell transplant. Blood Adv. 2020;4(14):3435-3442.
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Lívia Loss Sartori, Luiza Moreira da Silva Costa, Fernanda Miranda Prado, Lucas Brazolino Vianna Baptista, Letícia Loss Sartori, Síbia Soraya Marcondes, Ivie Moreira de Moura
This work is licensed under a Creative Commons Attribution 4.0 International License.
The Revista Brasileira de Pesquisa em Saúde (RBPS) adopts the CC BY 4.0 license, which means that authors retain the copyright of the works they submit to the journal. Authors are responsible for declaring that their contribution is an original manuscript, that it has not been previously published, and that it is not under simultaneous review by another scientific journal. Upon submitting the manuscript, authors grant RBPS the exclusive right of first publication, subject to peer review.
Authors are permitted to enter into additional contracts for the non-exclusive distribution of the version published by RBPS (for example, in institutional repositories or as a book chapter), provided that due acknowledgment of authorship and of initial publication by RBPS is given. Authors are also encouraged to make their work available online (for example, in institutional repositories or on their personal pages) after its initial publication in the journal, with due acknowledgment of authorship and of the original publication by RBPS.
Accordingly, under the CC BY 4.0 license, readers have the right to:
- Share — copy and redistribute the material in any medium or format for any purpose, even commercially;
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
The licensor cannot revoke these freedoms as long as you follow the license terms. Under the following terms:
- Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
