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10.1097/DAD.0000000000000348

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suck abstract from ncbi


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pmid26588336
      Am+J+Dermatopathol 2015 ; 37 (12 ): 924-8
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  • Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric Population: A Case Series and Review of the Literature #MMPMID26588336
  • Nguyen CM ; Stuart L ; Skupsky H ; Lee YS ; Tsuchiya A ; Cassarino DS
  • Am J Dermatopathol 2015[Dec]; 37 (12 ): 924-8 PMID26588336 show ga
  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy primarily found in adults, often carrying a poor prognosis. There are only 33 reported pediatric cases of BPDCN in the literature. Although standard treatment is not yet established for children, current literature recommends the use of high-risk acute lymphoblastic leukemia (ALL)-type chemotherapy. Recent studies, however, have explored the benefits of combining chemotherapy with stem-cell transplantation. Here, the authors present 2 cases of pediatric BPDCN treated with different modalities. The first case is a 13-year-old girl who presented with a 3-month history of an initially asymptomatic firm nodule on her left shin. The second case is a 15-year-old boy who presented with a 4-month history of an enlarging subcutaneous nodule on the lower leg. Immunohistochemical staining of both patients was positive for markers consistent with BPDCN. The latter patient received ALL-type therapy alone, whereas the former received ALL-type chemotherapy and stem-cell transplantation. Since initial treatment, both patients remain disease-free. These cases contribute to the limited number of pediatric BPDCN cases, thus helping to advance our knowledge toward an optimal treatment protocol for clinical remission.
  • |Adolescent [MESH]
  • |Antineoplastic Combined Chemotherapy Protocols/therapeutic use [MESH]
  • |Bone Marrow Transplantation [MESH]
  • |Dendritic Cells/*pathology [MESH]
  • |Female [MESH]
  • |Hematologic Neoplasms/*therapy [MESH]
  • |Hematopoietic Stem Cell Transplantation [MESH]
  • |Humans [MESH]
  • |Male [MESH]


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