Table 1.

Classification of T-cell lymphomas with primary cutaneous manifestations according to their clinical behavior, frequency and disease-specific 5-year survival. Based on the WHO-EORTC guidelines.¹

	Clinical behavior	Frequency (%)	Disease-specific 5-year survival (%)
Mycosis fungoides	Indolent	39	88
Mycosis fungoides variants
Folliculotropic mycosis fungoides	Indolent	5	75
Pagetoid reticulosis	Indolent	<1	100
Granulomatous slack skin	Indolent	<1	100
Sézary syndrome	Aggressive	2	36
Primary Cutaneous CD30+ Lymphoproliferative Disorders
Primary cutaneous anaplastic large T-cell lymphoma	Indolent	8	95
Lymphomatoid papulosis	Indolent	12	99
Adult T-cell lymphoma/leukemia	Indolent / Aggressive	<1	NR
Subcutaneous panniculitis-like T-cell lymphoma	Indolent	1	87
Extranodal NK/T-cell lymphoma, nasal type	Aggressive	<1	16
Chronic active EBV infection	Indolent	<1	NR
Primary cutaneous T-cell lymphoma γ/δ	Aggressive	<1	11
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma	Aggressive	<1	31
Primary cutaneous acral CD8+ T-cell lymphoma	Indolent	<1	100
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder	Indolent	6	100
Primary Cutaneous Peripheral T-Cell Lymphoma, NOS	Aggressive	2	15

NR, not reported; NOS, not otherwise specified.