Références

Ingen-Housz-Oro S, Beylot-Barry M. Que retenir de l'actualisation 2023 des recommandations EORTC pour le traitement du mycosis fongoïde et du syndrome de Sézary ? Ann Dermatol Venereol FMC. 2024;4(7):483-490. doi:10.1016/j.fander.2024.07.004.
Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023. Eur J Cancer. 2023;195:113343. doi:10.1016/j.ejca.2023.113343.
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G, Dummer R, Ranki A, Burg G, Heald P, Pittelkow M, Bernengo MG, Sterry W, Laroche L, Trautinger F, Whittaker S. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713-22. doi:10.1182/blood-2007-03-055749. PubMed PMID: 17540844.

Développé à des fins pédagogiques, ne se substitue pas à une décision médicale individualisée en RCP.

Classification TNMB

TNMB staging for mycosis fungoides and Sézary syndrome.

Skin
T1	Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque +/- patch).
T2	Patches, papules, or plaques covering ≥10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque +/- patch).
T3	One or more tumours (≥1-cm diameter)
T4	Confluence of erythema covering ≥80% body surface area
Node
N0	No clinically abnormal peripheral lymph nodes; biopsy not required
N1	Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0–2 N1a Clone negative N1b Clone positive
N2	Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3 N2a Clone negative N2b Clone positive
N3	Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3–4 or NCI LN4; clone positive or negative
Nx	Clinically abnormal peripheral lymph nodes; no histologic confirmation
Visceral
M0	No visceral organ involvement
M1	Visceral involvement (must have pathology confirmation and organ involved should be specified)
Blood*
B0	Absence of significant blood involvement: ≤5% of peripheral blood lymphocytes are atypical (Sézary) cells
B0a	Clone negative
B0b	Clone positive
B1	Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sézary) cells but does not meet the criteria of B2
B1a	Clone negative
B1b	Clone positive
B2	High blood tumour burden: ≥1000/μl Sézary cells with positive clone

SS is staged as T4 N2/3/x M0 B2.

* Blood staging for MF/SS is further defined as B0 = <250/ml of CD4+/CD26- or CD4+/CD7- cells, B1 = does not meet criteria for B0 or B2, and B2 = ≥1000/ml of CD4+/CD26- or CD4+/CD7- cells or other aberrant population of lymphocytes identified by flow cytometry [3], [240].

Outil de classification TNMB du mycosis fongoïde et du syndrome de Sézary

Cet outil interactif détermine le stade clinique ISCL/EORTC d'un mycosis fongoïde ou d'un syndrome de Sézary à partir de l'atteinte cutanée (T), ganglionnaire (N), viscérale (M) et sanguine (B). Il affiche la survie sans maladie (DSS) à 5 ans selon Olsen et al. et propose le bilan d'examens complémentaires adapté.

Développé à des fins pédagogiques pour les dermatologues et oncologues. Ne se substitue pas à une décision médicale individualisée en RCP.

Mycosis fongoïde / Syndrome de Sézary - TNMB

Peau

Ganglions

Métastases

Sang

Examens

Traitement

Références

Classification TNMB

Outil de classification TNMB du mycosis fongoïde et du syndrome de Sézary