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Mycosis Fungoides Cooperative Group TNMB classification of cutaneous T cell lymphoma (CTCL)

Mycosis Fungoides Cooperative Group TNMB classification of cutaneous T cell lymphoma (CTCL)
Skin (T)
T1 Limited patches*, papules, and/or plaques covering <10 percent of the skin surface; may further stratify into T1a (patch only) versus T1b (plaque ± patch)
T2 Patches, papules, or plaques covering ≥10 percent of the skin surface; may further stratify into T2a (patch only) versus T2b (plaque ± patch)
T3 One or more tumorsΔ (≥1 cm diameter)
T4 Confluence of erythema covering ≥80 percent body surface area
Node (N)
N0 No clinically abnormal lymph nodes; biopsy not required
N1 Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a Clone negative§
N1b Clone positive§
N2 Clinically abnormal lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a Clone negative§
N2b Clone positive§
N3 Clinically abnormal lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative
NX Clinically abnormal 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 No significant blood involvement: ≤5 percent of Sézary cells. For clinical trials, B0 may also be defined as <250/microL Sézary cells; CD4+CD26- or CD4+CD7- cells or CD4+CD26- and CD4+CD7- cells <15 percent by flow cytometry.
B0a Clone negative
B0b Clone positive
B1 Low blood tumor burden: Does not meet the criteria of B0 or B2
B1a Clone negative
B1b Clone positive
B2 High blood tumor burden: Positive cloneplus one of the following: ≥1000/microL Sézary cells; CD4/CD8 ≥10; CD4+CD7- cells ≥40 percent; or CD4+CD26- cells ≥30 percent. For clinical trials, B2 may also be defined as >1000/microL CD4+CD26- or CD4+CD7- cells.
* For skin, patch indicates any size lesion without significant elevation or induration. Presence/absence of hypo- or hyperpigmentation, scale, crusting, and/or poikiloderma should be noted.
¶ For skin, plaque indicates any size skin lesion that is elevated or indurated. Presence or absence of scale, crusting, and/or poikiloderma should be noted. Histologic features such as folliculotropism or large-cell transformation (>25 percent large cells), CD30+ or CD30-, and clinical features such as ulceration are important to document.
Δ For skin, tumor indicates at least one 1 cm diameter solid or nodular lesion with evidence of depth and/or vertical growth. Note total number of lesions, total volume of lesions, largest size lesion, and region of body involved. Also note if histologic evidence of large-cell transformation has occurred. Phenotyping for CD30 is encouraged.
For node, abnormal lymph node(s) indicates any lymph node that on physical examination is firm, irregular, clustered, fixed, or 1.5 cm or larger in diameter or on imaging is >1.5 cm in the long axis or >1 cm in the short axis. Node groups examined on physical examination include cervical, supraclavicular, epitrochlear, axillary, and inguinal.
§ A T cell clone is defined by polymerase chain reaction or Southern blot analysis of the T cell receptor gene.
¥ For viscera, spleen and liver may be diagnosed by imaging criteria alone.
‡ The clone in the blood should match that of the skin. The relevance of an isolated clone in the blood or a clone in the blood that does not match the clone in the skin remains to be determined.
This research was originally published in Blood. Olsen E, Vonderheid E, Pimpinelli N, et al. 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 2007; 110:1713. Copyright © the American Society of Hematology.
Additional data from: Olsen EA, Whittaker S, Kim YH, et al. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol 2011; 29:2598.
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