Aggressive epidermotropic cutaneous CD8⁺ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop

Abstract

Aggressive epidermotropic cutaneous CD8(+) lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas

“…CD8 positive aggressive epidermotropic T-cell lymphoma has been defined clinically by the abrupt presentation of extensive annular plaques with erosive features and ulceration (6, 7). This observation reflects the cytotoxic nature of the lymphoma cells which are embedded primarily in the intraepithelial compartments including epidermis and skin adnexa.…”

Significance of CD30 Expression by Epidermotropic T Cells in Lymphomatoid Papulosis and Lymphomatoid Pityriasis Lichenoides

“…The immunophenotype of these atypical cells was CD8+CD4-CD30+ indicating the possibility of aggressive primary cutaneous epidermotropic CD8+ cytotoxic T-cell lymphoma, also known as Berti's lymphoma. [10,11] The infiltrate was not angiocentric nor was vasculitis present, features characteristic of LyP-E. [12] Epidermotropic T cells were CD8+ and approximately one-half expressed of CD30. Although the patient's disease responded well to low doses of methotrexate, her disease would abruptly recur when methotrexate doses were reduced.…”

Complete durable remission of a fulminant primary cutaneous aggressive epidermotropic CD8 + cytotoxic T-cell lymphoma after autologous and allogeneic hematopoietic stem cell transplantation

“…The prognosis of aggressive epidermotropic CD8 + CTCL is poor, with median survival of 12 months 5 and average 5-year survival rate estimated to be 0% 1 to 18%, 6 mainly because of high aggressiveness; rapid spread to the lungs, testes, and central nervous system; and low response rate to chemotherapy. Nevertheless, systemic polychemotherapy is usually attempted but often without lasting response 6 .…”