title: Most primary cutaneous CD30-positive lymphoproliferative disorders have a CD4-positive cytotoxic T-cell phenotype.
abstract: Primary cutaneous CD30-positive (anaplastic) large T-cell lymphomas and lymphomatoid papulosis are closely related types of cutaneous T-cell lymphoma with a favorable prognosis. The neoplastic T cells in these conditions have the phenotype of activated CD3+, CD4+, CD8-, CD30+ skin homing T cells, but their normal counterpart has not yet been defined. To further characterize the cellular origin of the neoplastic T cells, skin biopsies from 14 patients with primary cutaneous CD30-positive (anaplastic) large T-cell lymphomas, nine patients with lymphomatoid papulosis, and six patients with primary cutaneous CD30-negative pleomorphic large T-cell lymphomas were stained with monoclonal antibodies against cytotoxic cell-associated molecules granzyme B and T-cell restricted intracellular antigen. In nine of nine lymphomatoid papulosis and in 10 of 14 CD30-positive primary cutaneous large T-cell lymphomas, expression of granzyme B and T-cell restricted intracellular antigen by variable numbers of neoplastic cells was found. Expression of granzyme B by the neoplastic CD30-positive T cells was confirmed by double-staining for granzyme B and CD30 (three cases) and by the detection of granzyme B mRNA using RNA in situ hybridization (one case). In most cases equal numbers of granzyme-B-positive and T-cell restricted intracellular antigen positive tumor cells were observed. In five of six CD30-negative primary cutaneous large T-cell lymphomas the neoplastic cells did not express these proteins, whereas in one case a sporadic positive tumor cell was found. These results demonstrate that, in contrast to primary cutaneous CD30-negative pleomorphic large T-cell lymphomas, the neoplastic cells in most primary cutaneous CD30-positive (anaplastic) large T-cell lymphomas and lymphomatoid papulosis have a unique CD4+, CD8-, cytotoxic T-cell phenotype.
authors.affiliation: Department of Pathology, Free University Hospital, Amsterdam, The Netherlands.
authors.forename: J A
authors.initials: JA
authors.lastname: Kummer
authors.forename: M H
authors.initials: MH
authors.lastname: Vermeer
authors.forename: D
authors.initials: D
authors.lastname: Dukers
authors.forename: C J
authors.initials: CJ
authors.lastname: Meijer
authors.forename: R
authors.initials: R
authors.lastname: Willemze
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000378:metabolism*; D016179:Granulocyte Colony-Stimulating Factor / Q000096:biosynthesis; D053804:Granzymes; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000276:immunology; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000276:immunology*; D008565:Membrane Proteins / Q000096:biosynthesis; D010641:Phenotype; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins / Q000096:biosynthesis; D012697:Serine Endopeptidases / Q000096:biosynthesis; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D017730:Ki-1 Antigen; D008565:Membrane Proteins; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D016179:Granulocyte Colony-Stimulating Factor; C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases