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From the Institute of Dermatologic Science and IRCCS
Ospedale Maggiore,*
Milan, Italy; the Department of
Dermatology,
Ospedale Busto Arsizio, Italy;
and the Departments of Dermatology and
Pathology,
Free University Hospital,
Amsterdam, The Netherlands
Cutaneous T cell lymphomas (CTCL) generally have the phenotype of
CD3+, CD4+, CD45RO+ memory T cells. CTCL expressing a
CD8+ T cell phenotype are extremely rare and ill-defined. To elucidate
whether these CD8+ CTCL represent a distinct disease entity,
the clinical, histological, and immunophenotypical
features of 17 CD8+ CTCL were reviewed. None of the 17 cases expressed
markers characteristic of natural killer cells or 
/
T cells. Nine
of 17 cases showed the characteristic clinical and histological
features as well as clinical behavior of well defined types of
CTCL, such as mycosis fungoides (2 cases), pagetoid
reticulosis (2 cases), lymphomatoid papulosis (2
cases), and CD30+ large T cell lymphoma (2
cases), all of which usually express a CD4+ T cell
phenotype, and 1 case of subcutaneous panniculitis-like T cell
lymphoma. The other 8 cases formed a homogeneous group showing a
distinctive set of clinicopathological and immunophenotypical
features, not consistent with that of other well defined types
of CTCL. Clinical characteristics included presentation with
generalized patches, plaques, papulonodules,
and tumors mimicking disseminated pagetoid reticulosis; metastatic
spread to unusual sites, such as the lung,
testis, central nervous system, and oral
cavity, but not to the lymph nodes; and an aggressive course
(median survival, 32 months). Histologically, these
lymphomas were characterized by band-like infiltrates consisting of
pleomorphic T cells or immunoblasts, showing a diffuse
infiltration of an acanthotic epidermis with variable degrees of
spongiosis, intraepidermal blistering, and necrosis.
The neoplastic cells showed a high Ki-67 proliferation index and
expression of CD3, CD8, CD7, CD45RA,
ßF1, and TIA-1 markers, whereas CD2 and CD5 were
frequently lost. Expression of TIA-1 pointed out that these lymphomas
are derived from a cytotoxic T cell subset. The results of this and
other studies reviewed herein suggest that these strongly
epidermotropic primary cutaneous CD8+ cytotoxic T cell lymphomas
represent a distinct type of CTCL with an aggressive clinical
behavior.
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