Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas -A distinct clinicopathological entity with an aggressive clinical behavior

Cutaneous T cell lymphomas (CTCL) generally have the phenotype of CD3+, CD4 +, CD45RO+ memory T cells. CTCL expressing a CD8+ T cell phenotype are extr emely rare and iii-defined, To elucidate whether these CD8+ CTCL represent a distinct disease entity, the clinical, histological, and immunophenotypic al features of 17 CD8+ CTCL were reviewed. None of the 17 cases expressed m arkers characteristic of natural killer cells or gamma/delta T cells. Nine of 17 cases showed the characteristic clinical and histological features as well as clinical behavior of well defined types of CTCL, such as mycosis f ungoides (2 cases), pagetoid reticulosis (2 cases), lymphomatoid papulosis (2 cases), and CD30+ large T cell lymphoma (2 cases), all of which usually express a CD4+ T cell phenotype, and 1 case of subcutaneous panniculitis-li ke T cell lymphoma. The other 8 cases formed a homogeneous group showing a distinctive set of clinicopathological and immunophenotypical features, not consistent with that of other well. defined types of CTCL, Clinical charac teristics included presentation with generalized patches, plaques, papulono dules, and tumors mimicking disseminated pagetoid reticulosis; metastatic s pread to unusual sites, such as the lung, testis, central nervous system, a nd oral cavity, but not to the lymph nodes; and an aggressive course (media n survival, 32 months). Histologically, these lymphomas were characterized by band-like infiltrates consisting of pleomorphic T cells or immunoblasts, showing a diffuse infiltration of an acanthotic epidermis with variable de grees of spongiosis, intraepidermal blistering, and necrosis. The neoplasti c cells showed a high Ki-67 proliferation index and expression of CD3, CD8, CD7, CD45RA, beta F1, and TIA-1 markers, whereas CD2 and CD5 were frequent ly lost. Expression of TIA-1 pointed out that these lymphomas are derived f rom a cytotoxic T cell subset. The results of this and other studies review ed herein suggest that these strongly epidermotropic primary cutaneous CD8 cytotoxic T cell lymphomas represent a distinct type of CTCL with an aggre ssive clinical behavior.