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 histologic involvement

Stage IIB: skin tumors present, no histologic involvement or erythroderma

Stage III: erythroderma present, no histologic involvement

Stage IVA: lymph node and histologic involvement

Stage IVB: visceral and histologic involvement

The International Society for Cutaneous Lymphomas (ISCL)/European Organization of Research and Treatment of Cancer revised clinical staging and classification of mycosis fungoides and Sézary syndrome[39]

Tumor 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 tumors 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; histopathologic involvement, no atypical lymphocytes

    • N1a: clone-negative

    • N1b: clone-positive

  • N2: clinically abnormal peripheral lymph nodes; histopathologic involvement, aggregates of atypical lymphocytes

    • N2a: clone-negative

    • N2b: clone-positive

  • N3: clinically abnormal peripheral lymph nodes; histopathologic 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 histologic involvement.

Metastatic stage (visceral)

  • M0: no visceral disease

  • M1: visceral disease.

Blood stage

  • B0: No hematologic involvement; <5% atypical or Sézary cells in peripheral blood

    • B0a: clone-negative

    • B0b: clone-positive

  • B1: Sézary count 5% or more of peripheral blood lymphocytes, but does not meet criteria of B2

    • B1a: clone-negative

    • B1b: clone-positive

  • B2: 1000/microliter 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 tumor-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].com.bmj.content.model.Caption@385ebb9f

Lund and Browder 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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