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Frequency and prognosis of main types of primary cutaneous lymphomas according to the 2018 update of the WHO-EORTC classification

Frequency and prognosis of main types of primary cutaneous lymphomas according to the 2018 update of the WHO-EORTC classification
Revised WHO classification 2018 Frequency (%)* Five-year DSS (%)
Cutaneous T cell and NK cell lymphomas
Mycosis fungoides 39 89
Mycosis fungoides variants and subtypes
  • Folliculotropic mycosis fungoides
5 79
  • Pagetoid reticulosis
<1 100
  • Granulomatous slack skin
<1 100
Sézary syndrome 2 30
Adult T cell leukemia/lymphoma <1 NDA
Primary cutaneous CD30+ lymphoproliferative disorders
  • Primary cutaneous anaplastic large cell lymphoma
10 95
  • Lymphomatoid papulosis
15 100
Subcutaneous panniculitis-like T cell lymphoma 1 87
Extranodal NK/T cell lymphoma, nasal type <1 NR
Primary cutaneous peripheral T cell lymphoma, rare subtypes
  • Primary cutaneous gamma-delta T cell lymphoma
<1 NR
  • Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma (provisional)
<1 12
  • Primary cutaneous CD4+ small/medium-sized pleomorphic T cell lymphoproliferative disorder (provisional)
3 100
  • Primary cutaneous acral CD8+ T cell lymphoma (provisional)
<1 100
Primary cutaneous peripheral T cell lymphoma, not otherwise specified 2 15
Cutaneous B cell lymphomas
Primary cutaneous marginal zone B cell lymphoma 7 99
Primary cutaneous follicle center lymphoma 12 95
Primary cutaneous diffuse large B cell lymphoma, leg type 4 52
WHO: World Health Organization; EORTC: European Organization for Research and Treatment of Cancer; DSS: disease-specific survival; NK: natural killer; NDA: no data available; NR: not reached.
* Relative frequency and disease-specific five-year-survival.
Adapted from: Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019; 133:1703.
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