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.

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.