Primary cutaneous peripheral T cell lymphoma, unspecified

Epidemiology:

•   primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):

Common Sites:

•  primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):

• skin
• may disseminate to visceral sites
• lymph nodes often spared

Gross features:

•   primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):

• localized or disseminated eruptive papules, nodules, and tumours
1. central ulceration and necrosis
• OR superficial hyperkeratotic patches and plaques

Histologic features:

•  primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):
• epidermotropic CD8+ cytotoxic T cells
• small-medium or medium-large
• pleomorphic or blastic nuclei
• acanthotic or atrophic epidermis
• necrotic keratinocytes
• ulceration
• variable spongiosis
• blister formation sometimes
• invasion and destruction of adnexal skin structures commonly
• angiocentricity / angioinvasion maybe

Immunophenotype:

Marker:	Sensitivity:	Specificity:
BetaF1
CD3
CD8
Granzyme B perforin
TIA-1
CD45RA
CD45RO (neg)
CD2 (neg)
CD4 (neg)
CD5 (neg)
CD7	+/-
EBV (neg)	“generally”

Molecular features:

•   primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):

• none specific

Other features:

•   primary cutaneous aggressive epidermotrophic CD8+ cytotoxic T cell lymphoma (provisional entity):

• aggressive clinical behaviour – median survival of 32 months

References:

• Willemze et al.  Blood, 15 May 2005 – Volume 105, number 10.