Table 1.

List of primary CTCLs.

Study group	Frequency, %			Disease-specific 5-year survival2, %
	DACLG2	SEER165	JSCS6
Mycosis fungoides	61.5	54.1	51.7	88
Sézary syndrome	3.5	1.2	2.3	24
Primary cutaneous CD30+ T-cell lymphoproliferative disorders	26.0	14.4	14.3
Lymphomatoid papulosis	16.1		4.5	100
Primary cutaneous anaplastic large-cell lymphoma	9.9		9.4	95
Adult T-cell leukemia/lymphomaa		0.1	20.0
Subcutaneous panniculitis-like T-cell lymphoma	1.2	0.8	2.3	82
Primary cutaneous gamma/delta T-cell lymphoma	0.9		0.3	NR
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorderb	2.7		1.7	75
Hydroa vacciniforme-like lymphoproliferative disorderb
Primary cutaneous acral CD8+ T-cell lymphomab
Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma	1.0		0.4	18
Peripheral T-cell lymphoma, NOSa	3.2	29.4	6.9	16
Total no. of CTCL	1,469	2,750	1,451

NR, not reached; CTCL, cutaneous T-cell lymphoma.

^aA portion of these diseases is considered as primary CTCL.

^bProvisional entity in World Health Organization classification (2016).