Criteria
Original clinical staging system for CTCL[39]
Stage IA: patches and/or plaques; <10% body surface area involved
Stage IB: patches and/or plaques; 10% or more of body surface area involved
Stage IIA: enlarged lymph nodes, no histological involvement
Stage IIB: skin tumours present, no histological involvement or erythroderma
Stage III: erythroderma present, no histological involvement
Stage IVA: lymph node and histological involvement
Stage IVB: visceral and histological involvement
The International Society for Cutaneous Lymphomas (ISCL)/European Organisation of Research and Treatment of Cancer revised clinical staging and classification of mycosis fungoides and Sézary syndrome[39]
Tumour stage (skin)
T1: limited patches, papules, and/or plaques covering <10% of skin surface
T1a: patch only
T1b: plaque with or without patch
T2: patches, papules, or plaques covering 10% or more of skin surface
T2a: patch only
T2b: plaque with or without patch
T3: one or more tumours equal to or greater than 1 cm diameter
T4: confluence of erythema, covering 80% or more of body surface area.
Nodal stage
N0: no clinically abnormal peripheral lymph nodes; biopsy not required
N1: clinically abnormal peripheral lymph nodes; histopathological involvement, no atypical lymphocytes
N1a: clone-negative
N1b: clone-positive
N2: clinically abnormal peripheral lymph nodes; histopathological involvement, aggregates of atypical lymphocytes
N2a: clone-negative
N2b: clone-positive
N3: clinically abnormal peripheral lymph nodes; histopathological involvement, partial or complete effacement of nodal architecture by atypical lymphocytes or neoplastic cells; clone-positive or -negative
Nx: clinically abnormal peripheral lymph nodes; no confirmed histological involvement.
Metastatic stage (visceral)
M0: no visceral disease
M1: visceral disease.
Blood stage
B0: No haematological involvement; <5% atypical or Sézary cells in peripheral blood
B0a: clone-negative
B0b: clone-positive
B1: Sézary cell count 5% or more of peripheral blood lymphocytes, but does not meet criteria of B2
B1a: clone-negative
B1b: clone-positive
B2: 1000/microlitre or more Sézary cells; clone-positive.
ISCL diagnostic criteria for early mycosis fungoides[39]
The ISCL recommends that patients with suspicious patch or plaque disease, or those with tumour-stage disease who do not meet the criteria laid out herewith, should not be entered into mycosis fungoides (MF) or Sézary syndrome (SS) databases or therapeutic trials for MF or SS. [Figure caption and citation for the preceding image starts]: Criteria for diagnosing early mycosis fungoidesAdapted from the International Society for Cutaneous Lymphomas (ISCL) algorithm for the diagnosis of early mycosis fungoides [Citation ends].
Lund and Browder's chart[40]
Extent of disease and response to therapy may be determined by using the assessment of percentage skin involvement initially devised by Lund and Browder.
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