ArticleThe American Journal of dermatopathology2025
Primary Cutaneous CD4+ Small/Medium-Sized T-Cell Lymphoproliferative Disorder: A Retrospective Cohort Study of 40 Adult Patients.
Article in The American Journal of dermatopathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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2 authors.
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Abstract
abstractPrimary cutaneous CD4+ small/medium-sized T-cell lymphoproliferative disorder (CD4+ PCSM-LPD) is distinguished from other peripheral T-cell lymphomas, particularly multifocal variants, by its indolent clinical behavior and favorable prognosis. Recent studies have suggested a T follicular helper (TFH) cell origin for these lesions; however, further studies are warranted to substantiate this hypothesis and clarify their pathogenesis. A retrospective review was conducted of all adult cases of CD4+ PCSM-LPD diagnosed at Weill Cornell Medicine between 2018 and 2024. The aim of this study was to evaluate the clinical, pathological, and molecular characteristics of these cases, with a specific focus on exploring the hypothesis of a follicular helper T-cell origin in CD4+ PCSM-LPD. Forty patients (26 men, 14 women) were encountered (age 25-83 years at presentation). Except for 1 oligolesional case, all others presented with solitary lesions, most frequently involving the head and neck region (26 of 37 cases, 72.2%). Treatment included surgical excision or radiation alone, with 1 lesion resolving spontaneously following the initial biopsy. Recurrence occurred only in 1 oligolesional case. All cases displayed characteristic histopathology of CD4+ PCSM-LPD. Varying positivity for nonspecific TFH markers (PD-1, BCL-6 and ICOS) was observed; both PD1 and ICOS can be expressed by activated T cells. Specific markers, CD10 and CXCL13, were predominantly negative the staining profile of CD4+ PCSM-LPD therefore suggests a partially developed TFH phenotype, reflecting the dynamic acquisition of these markers by neoplastic T cells in a conducive monocyte derived CD11c dendritic cell enriched microenvironment. Light chain restriction for plasma cells was observed in a qaurter of the cases and reflects the role of plasma cells as a countercheck population controlling follicular helper T cell expansion. Finally the lack of a follicular helper T cell phenotype in select cases that are otherwise typical for CD4+ PCSM-LPD should not exclude the diagnosis.
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