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Clinicopathological analysis of 17 primary cutaneous T-cell lymphoma of the ?? phenotype from Japan.

ABSTRACT: Primary cutaneous ?? T-cell lymphoma (PCGD-TCL) is an aggressive lymphoma consisting of clonal proliferation of mature activated ?? T-cells of a cytotoxic phenotype. Because primary cutaneous ?? T-cell lymphoma is a rare disease, there are few clinicopathological studies. In addition, T-cell receptor (TCR) ?? cells are typically immunostained in frozen sections or determined by TCR? negativity. We retrospectively analyzed 17 primary cutaneous T-cell lymphomas of the ?? phenotype (CTCL-??) in a clinicopathological and molecular study using paraffin-embedded sections. Among 17 patients, 11 had CTCL-?? without subcutaneous panniculitis-like T-cell lymphoma (SPTCL) features and six had CTCL-?? with SPTCL features. Immunophenotypically, some significant differences were found in CD8 and CD56 positivity between our patient series of CTCL-?? patients with SPTCL features and SPTCL-?? patients described in the previous literature. A univariate analysis of 17 CTCL-?? patients showed that being more than 60 years old, presence of visceral organ involvement, and small-to-medium cell size were poor prognostic factors. In addition, the 5-year overall survival rate was 42.4% for the CTCL-?? patients without SPTCL features and 80.0% for those with SPTCL features. Consequently, there was a strikingly significant difference in overall survival among SPTCL, CTCL-?? with SPTCL features and CTCL-?? without SPTCL features (P = 0.0005). Our data suggests that an indolent subgroup may exist in CTCL-??. Studies on more cases, including those from other countries, are warranted to delineate the clinicopathological features and the significance in these rare lymphomas.

SUBMITTER: Takahashi Y 

PROVIDER: S-EPMC4317912 | BioStudies | 2014-01-01T00:00:00Z

REPOSITORIES: biostudies

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