Sezary syndrome classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]
Overview
Sezary syndrome may be classified into subtypes based on TNMB. The staging of Sezary syndrome is based on the clinical stages.
Classification
- The staging of sezazry syndrome is based on the TNMB:[1]
- Sezary syndrome is defined by T4 erythroderma of body surface area (BSA) more than of 80 percent, Sezary cell is more than 1000 cells/microL in B2 involvement of peripheral blood staged of Sezary syndrome is based on the presence of nodal and/or visceral involvement[2]
| Staging for mycosis fungoides and Sezary syndrome | ||
|---|---|---|
| Skin (T) | ||
| 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 (N) | ||
| N0 | No clinically abnormal peripheral lymph nodes; biopsy not required | |
| N1 | Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2 | |
| N1a | Clone negative | |
| N1b | Clone posetive | |
| N2 | Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3 | |
| N2a | Clone negatove | |
| N2b | Clone posetive | |
| N3 | Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative | |
| NX | Clinically abnormal peripheral lymph nodes; no histologic confirmation | |
| Visceral (M) | ||
| M0 | No visceral organ involvement | |
| M1 | Visceral involvement (must have pathology confirmation and organ involved should be specified) | |
| Blood (B) | ||
| B0 | 0 Absence of significant blood involvement: 5% of peripheral blood lymphocytes are atypical (Sezary) cells B0a Clone negative B0b Clone positive | |
| B1 | Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive | |
| B2 | High blood tumour burden: 1000/mL Sezary cells with positive clone | |
The staging of Sezary syndrome is based on the clinical stages:[1][3]
| clinical stages | |||||
|---|---|---|---|---|---|
| Stage | T | N | M | B | DDS |
| IA | 1 | 0 | 0 | 0/1 | 98 |
| IB | 2 | 0 | 0 | 0/1 | 89 |
| IIA | 1.2 | 1.2 | 0 | 0/1 | 89 |
| IIB | 3 | 0-2 | 0 | 0/1 | 56 |
| IIIA | 4 | 0-2 | 0 | 0 | 54 |
| IIIB | 4 | 0-2 | 0 | 1 | 48 |
| IVA1 | 1-4 | 0-2 | 0 | 2 | 41 |
| IVA2 | 1-4 | 3 | 0 | 0-2 | 23 |
| IVB | 1-4 | 0-3 | 1 | 0-2 | 18 |
References
- ↑ 1.0 1.1 Trautinger, Franz; Eder, Johanna; Assaf, Chalid; Bagot, Martine; Cozzio, Antonio; Dummer, Reinhard; Gniadecki, Robert; Klemke, Claus-Detlev; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Pimpinelli, Nicola; Quaglino, Pietro; Ranki, Annamari; Scarisbrick, Julia; Stadler, Rudolf; Väkevä, Liisa; Vermeer, Maarten H.; Whittaker, Sean; Willemze, Rein; Knobler, Robert (2017). "European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017". European Journal of Cancer. 77: 57–74. doi:10.1016/j.ejca.2017.02.027. ISSN 0959-8049.
- ↑ 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 (September 2007). "Revisions to the staging and classification of mycosis fungoides and Sezary 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. 110 (6): 1713–22. doi:10.1182/blood-2007-03-055749. PMID 17540844.
- ↑ Jawed, Sarah I.; Myskowski, Patricia L.; Horwitz, Steven; Moskowitz, Alison; Querfeld, Christiane (2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)". Journal of the American Academy of Dermatology. 70 (2): 205.e1–205.e16. doi:10.1016/j.jaad.2013.07.049. ISSN 0190-9622.