Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma

Erythrodermic cutaneous T-cell lymphoma (CTCL) includes patients with eryth rodermic mycosis fungoides who may or may not exhibit blood involvement and Sezary syndrome and in whom hematological involvement is, by definition, p resent at diagnosis. These patients were stratified into 5 hematologic stag es (H0-H4) by measuring blood tumor burden, and these data were correlated with survival. The study identified 57 patients: 3 had no evidence of hemat ologic involvement (H0), 8 had a peripheral blood T-cell clone detected by polymerase chain reaction (PCR) analysis of the T-cell receptor gene and le ss than 5% Searzy cells on peripheral blood smear (H1), and 14 had either a T-cell clone detected by Southern blot analysis or PCR positivity with mor e than 5% circulating Sezary cells (H2), Twenty-four patients had absolute Sezary counts of more than 1 x 10(9) cells per liter (H3), and 8 patients h ad counts in excess of 10 x 10(9) cells per liter (H4), The disease-specifi c death rate was higher with increasing hematologic stage, after correcting for age at diagnosis. A univariate analysis of 30 patients with defined ly mph node stage found hematologic stage (P = .045) and lymph node stage (P = .013) but not age (P = .136) to be poor prognostic indicators of survival. Multivariate analysis identified only lymph node stage to be prognosticall y important, although likelihood ratio tests indicated that hematologic sta ge provides additional information (P = .035), Increasing tumor burden in b lood and lymph nodes of patients with erythrodermic CTCL was associated wit h a worse prognosis,The data imply that a hematologic staging system could complement existing tumor-node metastasis staging criteria in erythrodermic CTCL, (C) 2001 by The American Society of Hematology.