[Hematodermic CD4+ CD56+ neoplasm in childhood].
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.
Bilbao
Erica A Rojas
EA
Departamento de Patología, Instituto de Oncologia Angel H. Roffo, Universidad de Buenos Aires, Argentina.
Chirife
Ana María
AM
Florio
Darío
D
Giménez
Liliana B
LB
Marino
Lina
L
Rosso
Diego A
DA
D014408:Biomarkers, Tumor; D015704:CD4 Antigens; D019002:CD56 Antigen; D002648:Child; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D053650:Interleukin-3 Receptor alpha Subunit / Q000032:analysis; D007694:Killer Cells, Natural / Q000276:immunology; D037181:Lectins, C-Type / Q000032:analysis; D008223:Lymphoma / Q000276:immunology / Q000473:pathology*; D008562:Membrane Glycoproteins / Q000032:analysis; D011971:Receptors, Immunologic / Q000032:analysis; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
D014408:Biomarkers, Tumor; D015704:CD4 Antigens; D019002:CD56 Antigen; C443299:CLEC4C protein, human; D053650:Interleukin-3 Receptor alpha Subunit; D037181:Lectins, C-Type; D008562:Membrane Glycoproteins; D011971:Receptors, Immunologic