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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly identified, very rare, highly aggressive hematopoietic neoplasm, primarily found in elderly males. They typically present in the form of skin involvement with a high frequency of lymph node and bone marrow involvement. BPDCN has a very poor prognosis, with no consensus on a widely accepted treatment modality. Here we present a very young patient with BPDCN, who presented with generalized lymphadenopathy, skin involvement, and leukemic blasts in the bone marrow. She was treated with high-risk acute lymphocytic leukemia protocol, followed by allogeneic hematopoietic stem-cell transplantation, and has been in clinical remission for 12 months.
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Tekkeşin et al. (2020) studied this question.
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