Korean J Dermatol.  2013 Dec;51(12):970-974.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in Child

• Choi JH
• Kim BS
• Sohng SH
• Shin DH
• Choi JS

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.

Keyword

Blastic NK-cell lymphoma; Blastic plasmacytoid dendritic cell neoplasm; CD4+/CD56+ hematodermic neoplasm

MeSH Terms

Adult
Biopsy
Bone Marrow
Cheek
Child*
Dendritic Cells*
Dermis
Female
Hematologic Neoplasms
Herpesvirus 4, Human
Humans
Leukemia, Myeloid, Acute
Lymphocytes
Lymphoma
Purpura