Update on erythrodermic cutaneous T-cell lymphoma: Report of the International Society for Cutaneous Lymphomas

Eric C. Vonderheid, Maria Grazia Bernengo, Günter Burg, Madeleine Duvic, Peter Heald, Liliane Laroche, Elise Olsen, Mark Pittelkow, Robin Russell-Jones, Masahiro Takigawa, Rein Willemze

Research output: Contribution to journalArticlepeer-review

Abstract

Two conferences were sponsored by the International Society for Cutaneous Lymphomas (ISCL) to gain consensus on definitions and terminology for clinical use in erythrodermic cutaneous T-cell lymphoma (E-CTCL). Three subsets of E-CTCL were defined: Sézary syndrome ("leukemic phase" E-CTCL), erythrodermic mycosis fungoides (secondary E-CTCL that develops in patients with mycosis fungoides), and E-CTCL, not otherwise defined. The hematologic criteria recommended for Sézary syndrome are intended to identify patients with a worse prognosis compared with the other E-CTCL subsets and consist of one or more of the following: (1) an absolute Sézary cell count of 1000 cells/mm3 or more; (2) a CD4/CD8 ratio of 10 or higher caused by an increase in circulating T cells and/or an aberrant loss or expression of pan-T cell markers by flow cytometry; (3) increased lymphocyte counts with evidence of a T-cell clone in the blood by the Southern blot or polymerase chain reaction technique; or (4) a chromosomally abnormal T-cell clone. For staging purposes, it is proposed that these criteria define the B2 blood rating and that the B2 rating be considered equivalent to nodal involvement.

Original languageEnglish (US)
Pages (from-to)95-106
Number of pages12
JournalJournal of the American Academy of Dermatology
Volume46
Issue number1
DOIs
StatePublished - 2002
Externally publishedYes

ASJC Scopus subject areas

  • Dermatology

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