251. [Hematodermic CD4+ CD56+ neoplasm in childhood].
- Author
-
Bilbao EA, Chirife AM, Florio D, Giménez LB, Marino L, and Rosso DA
- Subjects
- Child, Dendritic Cells immunology, Dendritic Cells pathology, Diagnosis, Differential, Female, Flow Cytometry, Humans, Interleukin-3 Receptor alpha Subunit analysis, Killer Cells, Natural immunology, Lectins, C-Type analysis, Lymphoma immunology, Membrane Glycoproteins analysis, Receptors, Immunologic analysis, Skin Neoplasms immunology, Biomarkers, Tumor, CD4 Antigens, CD56 Antigen, Lymphoma pathology, Skin Neoplasms pathology
- Abstract
Hematodermic CD4+ CD56+ neoplasm with plasmacytoid dendritic cell phenotype is a rare and aggressive neoplasm recently recognized by the WHO-EORTC classification. It generally appears in elderly adults, exceptionally in childhood. We present a 12-year-old girl with severe mental retardation, genetic clinical features and multiple nodular cutaneous lesions on legs and arms. Histologically the nodules showed diffuse dermal infiltrate of medium and small cells and expression of CD4, CD56, CD43, S100 and plasmacytoid dendritic markers: CD123, BDCA-2 under flow cytometry study. Peripheral blood and bone marrow were not involved. Clinical remission of cutaneous lesions was observed after two weeks of acute lymphoblastic leukemia therapy.
- Published
- 2008