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Table 21-1

CTCL

MF

MF variants

  • Folliculotropic MF
  • Pagetoid reticulosis
  • Granulomatous slack skin

Sézary syndrome

Adult T-cell leukemia/lymphoma

Primary cutaneous CD30+ lymphoproliferative disorders

  • Cutaneous anaplastic large cell lymphoma
  • Lymphomatoid papulosis

Subcutaneous panniculitis-like T-cell lymphoma

Extranodal NK-T-cell lymphoma

Chronic active EBV infection

Primary cutaneous peripheral T-cell lymphoma

  • Primary cutaneous γ/δ T-cell lymphoma
  • CD8+ cutaneous aggressive epidermotropic T-cell lymphoma (provisional)
  • Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional)
  • Primary cutaneous acral CD8+ T-cell lymphoma (provisional)

Primary cutaneous peripheral T-cell lymphoma, NOS

CBCL

  • Primary cutaneous Marginal zone lymphoma
  • Primary cutaneous follicular center lymphoma
  • Primary cutaneous diffuse large B-cell lymphoma, leg type
  • EBV + mucocutaneous ulcer (provisional)
  • Intravascular large B-cell lymphoma

Source: Reproduced with permission from Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019; 133(16):1703-1714. Copyright © 2019 American Society of Hematology.