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KMID : 0363220130510120970
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Korean Journal of Dermatology
2013 Volume.51 No. 12 p.970 ~ p.974
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A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in Child
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Choi Jin-Hwa
Kim Byeong-Su
Sohng Seung-Hyun
Shin Dong-Hoon
Choi Jong-Soo
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Abstract
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematopoietic malignancywhich is derived from the precursors of plasmacytoid dendritic cells and is more infrequent in children than in adults.
Formerly known as blastic NK-cell lymphoma or CD4+/CD56+ hematodermic neoplasm, the BPDCN isreclassified into the group of acute myeloid leukemia and related neoplasm by WHO in 2008. An 8-year old girlis being presented with bruise-like subcutaneous nodules with purpura on her right cheek from the performed biopsy.
Histological examinations show sheet-like dense infiltrations of medium-sized lymphoid cells with irregularnuclei in the entire dermis. Immunohistochemical stainings of tumor cells were positive for CD4, CD56, LCA,TCL-1, TdT and focal positive for CD3, CD7, CD45RO and negative for CD20, CD30, CD34, EBV. The PET-CTscans indicate hot uptakes in the bone marrows which are suggestive of malignant infiltrations, and bone marrowbiopsy findings are consistent with BPDCN of leukemic transformations. We present a rare case of BPDCN whichaffects the pediatric patient.
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KEYWORD
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Blastic NK-cell lymphoma, Blastic plasmacytoid dendritic cell neoplasm, CD4+/CD56+ hematodermic neoplasm
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