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Blastic plasmacytoid dendritic cell neoplasm: an early presentation
Samira Oliveira Silveira, Cassia Michelle Almeida Fernandes1, Érica Baptista Pinto
1Pará State University, College of Medicine, Pará. cmich.fernandes@gmail.com.
Insights
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive skin cancer. This case highlights its rapid progression and fatal outcome, emphasizing the need for effective treatment strategies.
Area of Science:
- Dermatology
- Oncology
- Hematology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy primarily affecting the skin.
- It originates from plasmacytoid dendritic precursor cells and is characterized by rapid clinical progression.
Observation:
- A 46-year-old woman presented with a painless, pruritic nodule on her back, which rapidly grew and spread over six months.
- Histopathology revealed atypical lymphoid cell infiltrates in the dermis, with immunohistochemistry positive for CD45, S-100, CD123, and TCL1.
- The patient developed dyspnea and died from cardiorespiratory arrest within 12 hours of hospital admission.
Findings:
- The case demonstrates the aggressive nature of BPDCN, with rapid progression from cutaneous lesions to systemic compromise.
- Immunohistochemical markers confirmed the diagnosis of BPDCN.
Implications:
- There is currently no established consensus for BPDCN treatment.
- Aggressive therapeutic approaches, such as intensive leukemia-like therapy and allogeneic bone marrow transplantation, may offer improved long-term survival chances.
Abstract:
A blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a cutaneous lymphoma derived from a plasmacytoid dendritic precursor cell that exhibits aggressive clinical behavior. Herein, we report a 46-year-old woman with a complaint of a painless nodule on the back, associated with pruritus. The nodule grew and new growths appeared over six months of evolution. The histopathological examination of one of the left upper limb lesions showed a dense lymphoid cell infiltrate with atypia in the superficial and deep dermis. Immunohistochemistry showed positivity for CD45, S-100 protein, CD123, and TCL 1. About two months after the initial evaluation, the patient was admitted to the Emergency Hospital of Marituba-PA with dyspnea. She progressed to cardiorespiratory arrest and death within 12 hours of admission. There is still no consensus for the treatment of BPDCN. Intensive therapy for acute leukemia can be useful, but allogeneic bone marrow transplantation has a greater chance of long-term survival.
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