Long‐term follow‐up and management of small and medium‐sized CD4⁺ T cell lymphoma and CD8⁺ lymphoid proliferations of acral sites: a multicenter experience

Abstract

Background Primary cutaneous CD4+ small–medium pleomorphic T cell lymphoma (SMPTCL) is a low‐grade cutaneous T cell lymphoma. Its clinical and histopathologic features are comparable with those of CD8+ lymphoid proliferations (LPs) of the ear and acral sites. Objectives We performed a retrospective analysis of patients with CD4+ SMPTCL or CD8+ LP to elucidate the clinical course, prognosis, and outcomes. Methods Demographic, clinical, and treatment data were reviewed. Histopathologic data based on architectural, cytomorphologic, and immunohistochemical features were assessed. Immunohistochemical staining for T and B cell markers was evaluated. Results A total of 25 patients including 22 with CD4+ SMPTCL and three with CD8+ LP were identified. All patients presented with a single lesion, predominantly on the head, neck, or upper trunk (84%). No patients showed extracutaneous disease at any evaluation. The most common histopathologic changes showed a dense nodular infiltrate of small cells with hyperchromatic nuclei without significant follicular or adnexal involvement. Patients were treated with excision (48%), local radiation (28%), or topical or intralesional steroids (24%). All patients achieved complete resolution of disease. Five patients demonstrated cutaneous relapse at new sites. Conclusions The CD4+ SMPTCL/CD8+ LP subgroup usually presents with solitary lesions and demonstrates an indolent clinical course. Typical presentation, classic histopathology, widespread expression of follicular T helper cell markers, and loss of a T cell antigen are diagnostic features of CD4+ SMPTCL, whereas monomorphous CD8+ infiltrate without follicular T helper cell markers is consistent with CD8+ LP. Local skin‐directed therapy is appropriate in these patients.

Lymphoma classification update: T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms

“…The classic histopathologic changes include dermal infiltration by small lymphocytes with hyperchromatic nuclei, without significant epidermal, follicular, or adnexal involvement. Most cases have an indolent course and favorable outcome following excision, radiation, and/or other local therapies [2,54–56]. …”

Acral angiokeratoma‐like pseudolymphoma in a middle‐aged woman

“…Recently, the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms modified the terminology of primary cutaneous CD4+ small/medium‐sized pleomorphic T‐cell lymphoma into primary cutaneous CD4+ SMPTC‐LPD to reflect the limited clinical risk and uncertain significance of a malignant diagnosis in this localized disease . CD4+ SMPTC‐LPD presents as a solitary nodule or plaque in most of the cases, located mostly on the head and neck or upper trunk, and has an indolent clinical course . Clinically, the morphology and distribution of two adjacent scaly vascular papules localized to the toe are very unusual for SMPTC‐LPD.…”

Primary cutaneous CD4+ small‐/medium‐sized T‐cell lymphoproliferative disorder: A case report

“…Primary cutaneous CD4+ small‐/medium‐sized T‐cell lymphoproliferative disorder (PCSM‐TCLPD) is a rare and heterogeneous entity, with suggested derivation from follicular T‐helper lymphocytes. Most of the cases present as a solitary lesion in the upper part of the body and good response to surgical excision or local radiotherapy . The report describes a case of this rare disease with some peculiarities compared the typical described forms.…”