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.
Subject(s)
Adult , Child , Female , Humans , Biopsy , Bone Marrow , Cheek , Dendritic Cells , Dermis , Hematologic Neoplasms , Herpesvirus 4, Human , Leukemia, Myeloid, Acute , Lymphocytes , Lymphoma , PurpuraABSTRACT
CD4+/CD56+ hematodermic neoplasm is a rare and aggressive lesion that affects many organs, and skin involvement is highly characteristic. It is also termed blastic natural killer cell lymphoma in the World Health Organization classification. Several origins of tumor cells have been proposed, but recent studies have shown a relationship with plasmacytoid dendritic cells. A 2-year-old boy presented with multiple bruise-like violaceous subcutaneous nodules and plaques on the trunk, upper and lower extremities. Histological examination showed small-to-medium-sized blastoid cellular infiltration in the dermis and subcutaneous tissue. Tumor cells were positive for CD4, CD56 and TdT, and negative for CD8, CD20 and MPO. It primarily affects elderly patients, but, in this case, occurred in an infant. Due to its rarity, we present a case of CD4+/CD56+ hematodermic neoplasm affecting a pediatric patient.
Subject(s)
Aged , Humans , Infant , Dendritic Cells , Dermis , Killer Cells, Natural , Lower Extremity , Lymphoma , Child, Preschool , Skin , Subcutaneous Tissue , World Health OrganizationABSTRACT
CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is a rare and aggressive neoplasm with a high incidence of cutaneous involvement, risk of leukemic dissemination and poor prognosis. The characteristic features are expression of the T helper inducer cell marker CD4 and the NK-cell marker CD56 in the absence of other T cell or NKcell specific markers. Because of the rarity of this disease, we describe a 48 year old woman suffering from CD4+/CD56+ hematodermic neoplasm on her cheek without leukemic infiltration.