title: Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas.
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.
authors.affiliation: MCP Hahnemann University, Philadelphia, Pennsylvannia 19102, USA. vonder@erols.com
authors.forename: Eric C
authors.initials: EC
authors.lastname: Vonderheid
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Bernengo
authors.forename: Günter
authors.initials: G
authors.lastname: Burg
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
authors.forename: Peter
authors.initials: P
authors.lastname: Heald
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
authors.forename: Elise
authors.initials: E
authors.lastname: Olsen
authors.forename: Mark
authors.initials: M
authors.lastname: Pittelkow
authors.forename: Robin
authors.initials: R
authors.lastname: Russell-Jones
authors.forename: Masahiro
authors.initials: M
authors.lastname: Takigawa
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
mesh_terms: D003873:Dermatitis, Exfoliative; D003880:Dermatology; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous; D012878:Skin Neoplasms; D012955:Societies, Medical; D009626:Terminology as Topic