title: CD4/CD8 double negative pagetoid reticulosis: a case report and literature review.
abstract: Pagetoid reticulosis is an indolent primary cutaneous T-cell lymphoma. It typically presents as a solitary and slowly growing patch or plaque on the extremity, histologically characterized by an acanthotic epidermis infiltrated with atypical lymphocytes. Here, we present histological, immunophenotypical and molecular findings of a 29-year-old Jamaican man with bilateral wrist plaques. Histology showed marked acanthosis, hyperkeratosis and an intraepidermal infiltration consisting of large atypical lymphocytes. Immunohistochemical stains showed CD3 and CD5 positive T cells with significant loss of CD7, double negative CD4 and CD8 and strong positive CD30. Molecular analysis showed a monoclonal T-cell receptor (TCR) gamma gene rearrangement. Review of the literature confirms that the immunophenotype of pagetoid reticulosis is variable with decreasing frequency of CD8+ cytotoxic/suppressor T cell, CD4+ helper T cell and least commonly CD4/CD8 double negative phenotypes. Although CD4/CD8 double negative phenotype appears to be associated with higher proliferation index, it does not appear to confer prognostic significance.
authors.affiliation: Department of Pathology, George Washington University Hospital, Washington, DC, USA.
authors.forename: Nikki
authors.initials: N
authors.lastname: Mourtzinos
authors.forename: Puja K
authors.initials: PK
authors.lastname: Puri
authors.forename: Guanghua
authors.initials: G
authors.lastname: Wang
authors.forename: Min-Ling
authors.initials: ML
authors.lastname: Liu
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000276:immunology; D014408:Biomarkers, Tumor / Q000276:immunology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology* / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008297:Male; D056267:Pagetoid Reticulosis / Q000276:immunology* / Q000473:pathology / Q000532:radiotherapy; D012878:Skin Neoplasms / Q000276:immunology* / Q000473:pathology / Q000532:radiotherapy; D016896:Treatment Outcome
chemical_list: D015703:Antigens, CD; D014408:Biomarkers, Tumor