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.

CD4/CD8 Double Negative Mycosis Fungoides With PD-1 (CD279) Expression—A Disease of Follicular Helper T-Cells?

“…[10][11][12][13][14][15] Less than 35 cases of CD4/ CD8 double negative early MF have been reported since the first description in 1993 by Ralfkiaer and coworkers 3,[16][17][18][19] Our case supports the observation of Hodak et al that CD4/CD8 double negative MF is often associated with an unusual clinical presentation as in our patient in whom CD4/CD8 double negative MF clinically presented with annular and centrally hypopigmented lesions confined to the left lower leg as 1 body region also referred to as localized MF. Except for the expression of CD30 in early MF, none of the phenotypic MF variants seems to be of prognostic significance.…”

Woringer-Kolopp (Pagetoid Reticulosis) disease successful response to bexarotene gel

“…Histological studies demonstrate an acanthotic epidermis infiltrated by atypical lymphocytes with pagetoid spread . Previous studies have established a T‐cell lineage and clonality of the atypical lymphocytes . In contrast to classical MF, T cells in WK disease have a variable immunophenotype including CD8+ cytotoxic/suppressor, CD4+ helper, and rarely CD4/CD8 double negative phenotypes .…”

“…WK disease, first described in 1939, usually presents with a slow growing, solitary patch, or plaque on the extremities . The disease has been reported to have a predilection for middle‐age males.…”

Primary Cutaneous CD4/CD8–/– TCRαβ T-cell Lymphoma

“…The majority of cases of cutaneous CD4/CD8 -/-T-cell lymphoma are T-cell receptor (TCR) γδ type, which is classified as primary cutaneous γδ T-cell lymphoma in the latest WHO/EORTC classification (2). Cutaneous TCRαβ T-cell lymphoma with CD4/CD8 -/-phenotype is rare, and its origin has not been widely researched (3)(4)(5). We present here a case of cutaneous TCRαβ T-cell lymphoma with CD4/CD8 -/-phenotype with a unique clinical presentation, massive epidermotropism, and aggressive clinical course.…”