Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas - 01/09/11
Prepared on behalf of the ISCL by
Eric C. Vonderheid, MDa, Maria Grazia Bernengo, MDb, Günter Burg, MDc, Madeleine Duvic, MDd, Peter Heald, MDe, Liliane Laroche, MDf, Elise Olsen, MDg, Mark Pittelkow, MDh, Robin Russell-Jones, MDi, Masahiro Takigawa, MDj, Rein Willemze, MDkAbstract |
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. (J Am Acad Dermatol 2002;46:95-106.)
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Reprint requests: Eric Vonderheid, MD, MCP Hahnemann University, MS 478, Broad and Vine Streets, Philadelphia, PA 19102. E-mail: vonder@erols.com. |
Vol 46 - N° 1
P. 95-106 - janvier 2002 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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