Primary cutaneous acral CD8+ T-cell-lymphoma

Primary cutaneous acral CD8+ T-cell lymphoma is an entity first described in 2005 that presents with slow-growing papules and nodules mostly in - name-acral localization (Ally MS et al. 2014; Petrella T et al. 2007; Suchak R et al. 2010), shows indolent clinical behavior and no evidence of systemic involvement (Li J Y et al. 2014).

Although the majority of lesions are solitary and localized to the ears (Beltraminelli H et al. 2010; PetrellaT et al. 2007; Suchak R et al. 2010), bilateral symmetric presentations (Beltraminelli H et al. 2010) Petrella T et al., 2007;) Lesions on the nose (Greenblatt D et al. 2013; Suchak R et al. 2010) or on extrafacial, but mostly also acral localizations (Greenblatt D et al. 2013) have been described.

Dense diffuse dermal lymphoma infiltrate of medium sized CD8+ lymphocytes (Beltraminelli H et al. 2010) where the infiltrate usually shows a clear border zone to the overlying epithelium and has a low proliferation index (Li et al., 2014). The atypical T cells usually express CD3, CD8 and TIA1 and are negative for CD4, CD30, CD56 (Beltraminelli H et al. 2010).

While acral CD8+ cutaneous lymphoma was initially considered a CD8+ 6 variant of CD4+ small-medium cell TCL and not a distinct subgroup (Beltraminelli H et al. 2010), acral CD8+ TCL has since been included as a separate provisional entity in the 2016 WHO classification (Swerdlow et al., 2016)

1. Ally MS et al (2014) A review of the solitary cutaneous T-cell lymphomas. J Cutan Pathol 41: 703-714.
2. Beltraminelli H et al (2010) Indolent CD8+ lymphoid proliferation of the ear: a phenotypic variant of the small-medium pleomorphic cutaneous T-cell lymphoma? J Cutan Pathol 37: 81-84.
3. Greenblatt D et al (2013). Indolent CD8(+) lymphoid proliferation of acral sites: a clinicopathologic study of six patients with some atypical features. J Cutan Pathol 40: 248-258.
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