A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in Child.
- Author:
Jin Hwa CHOI
1
;
Byeong Su KIM
;
Seung Hyun SOHNG
;
Dong Hoon SHIN
;
Jong Soo CHOI
Author Information
1. Department of Dermatology, College of Medicine, Yeungnam University, Daegu, Korea. dhshin@med.yu.ac.kr
- Publication Type:Case Report
- Keywords:
Blastic NK-cell lymphoma;
Blastic plasmacytoid dendritic cell neoplasm;
CD4+/CD56+ hematodermic neoplasm
- MeSH:
Adult;
Biopsy;
Bone Marrow;
Cheek;
Child*;
Dendritic Cells*;
Dermis;
Female;
Hematologic Neoplasms;
Herpesvirus 4, Human;
Humans;
Leukemia, Myeloid, Acute;
Lymphocytes;
Lymphoma;
Purpura
- From:Korean Journal of Dermatology
2013;51(12):970-974
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematopoietic malignancy which 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 is reclassified into the group of acute myeloid leukemia and related neoplasm by WHO in 2008. An 8-year old girl is 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 irregular nuclei 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-CT scans indicate hot uptakes in the bone marrows which are suggestive of malignant infiltrations, and bone marrow biopsy findings are consistent with BPDCN of leukemic transformations. We present a rare case of BPDCN which affects the pediatric patient.
