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Diffuse lung metastases in a child with blastic plasmacytoid dendritic cell neoplasm and review

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Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly aggressive hematopoietic malignancy, characterized by cutaneous and bone marrow involvement and leukemic spread. Cutaneous involvement is the most common presentation in BPDCN. At present, the diagnosis and management of BPDCN are still challenging. Due to its rarity, the pediatric experience with BPDCN is especially limited. Herein, we report a special case of BPDCN with diffuse nodular lung metastases and a cutaneous lesion, which achieved a dramatic response to non-Hodgkin lymphoma regimen and remained with complete remission for 2 years. To date, acute lymphoblastic leukemia (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (SCT) is commonly thought to be related to a favorable outcome in adults with BPDCN. In contrast to it, ALL or non-Hodgkin lymphoma-type therapy alone seems enough in children with BPDCN with or without cutaneous lesions. SCT should only be performed for children who relapse and achieve a second remission. For pediatric BPDCN, further study of larger numbers of cases is needed to better define prognostic factors and optimal treatment strategy, and understand the underling differences in pathogenesis between children and adults.

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The authors indicated no potential conflicts of interest.

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Authors and Affiliations

  1. The Department of Pediatric Hematology and Oncology, the First Hospital of Jilin University, MD 71 Xinmin Street, Changchun, 130021, Jilin, China

    Xiao-dan Zhong, Li-zhe Wang, Xiao Wang & Jian Chang

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  1. Xiao-dan Zhong
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  2. Li-zhe Wang
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  3. Xiao Wang
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  4. Jian Chang
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Correspondence to Jian Chang.

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Zhong, Xd., Wang, Lz., Wang, X. et al. Diffuse lung metastases in a child with blastic plasmacytoid dendritic cell neoplasm and review. Eur J Pediatr 173, 1667–1670 (2014). https://doi.org/10.1007/s00431-013-2248-4

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  • DOI: https://doi.org/10.1007/s00431-013-2248-4

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