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Blood, 1 February 2001, Vol. 97, No. 3, pp. 624-630
CLINICAL OBSERVATONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Prognostic significance of tumor burden in the blood of patients
with erythrodermic primary cutaneous T-cell lymphoma
Julia J. Scarisbrick,
Sean Whittaker,
Alun V. Evans,
Elisabeth A. Fraser-Andrews,
Fiona J. Child,
Alan Dean, and
Robin Russell-Jones
From the Skin Tumour Unit, St John's Institute of
Dermatology, St Thomas' Hospital, London, England, and the Norfolk and
Norwich Hospital, Norfolk and Norwich NHS Trust, Norwich, England.
Erythrodermic cutaneous T-cell lymphoma (CTCL) includes patients
with erythrodermic mycosis fungoides who may or may not exhibit blood
involvement and Sézary syndrome and in whom hematological involvement is, by definition, present at diagnosis. These
patients were stratified into 5 hematologic stages (H0-H4) by measuring blood tumor burden, and these data were correlated with survival. The
study identified 57 patients: 3 had no evidence of hematologic involvement (H0), 8 had a peripheral blood T-cell clone detected by
polymerase chain reaction (PCR) analysis of the T-cell receptor gene
and less than 5% Sézary cells on peripheral blood smear (H1),
and 14 had either a T-cell clone detected by Southern blot analysis or
PCR positivity with more than 5% circulating Sézary cells (H2).
Twenty-four patients had absolute Sézary counts of more than
1 × 109 cells per liter (H3), and 8 patients had counts
in excess of 10 × 109 cells per liter (H4). The
disease-specific death rate was higher with increasing hematologic
stage, after correcting for age at diagnosis. A univariate analysis of
30 patients with defined lymph 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 prognostically important, although likelihood ratio tests indicated that hematologic stage provides additional information (P = .035). Increasing tumor burden in blood and lymph
nodes of patients with erythrodermic CTCL was associated with a worse
prognosis.The data imply that a hematologic staging system
could complement existing tumor-node-metastasis staging
criteria in erythrodermic CTCL.
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