CD8+ cutaneous anaplastic large-cell lymphoma: report of two cases with immunophenotyping, T-cell-receptor gene rearrangement and electron microscopic studies

Abstract

Two cases are reported of cutaneous anaplastic large-cell lymphoma with the suppressor/cytotoxic (CD8) phenotype. In both cases there was a solitary skin tumour in which there was a dense infiltrate with large irregularly shaped cells which on immunophenotyping expressed CD8. DNA hybridization analysis showed rearrangements of the T-cell-receptor gene in both cases.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

“…As many as 50% of cases of pagetoid reticulosis may have CD8 positivity, and more rarely, sporadic cases of MF, SS, and primary cutaneous CD30 1 lymphoproliferative disorders, including primary cutaneous anaplastic large-cell lymphoma and lymphomatoid papulosis, subcutaneous panniculitis-like T-cell lymphoma, and other forms of CTCL of unspecified type, may express CD8 1 phenotype. 1,2,4,8,11,20 These CD8 1 cases generally follow an indolent clinical course similar to that of the more common, classic CD4 1 variants. 1,2,5,8,20,21 The CD8 1 immunophenotypic variant of MF, for example, reported by Dummer et al, 21 is characterized by a slowly progressing course, responsiveness to initial treatment with nontoxic, conservative therapy including topical corticosteroids and UVB or PUVA, similar to cases of classic MF.…”

“…1,2,4,8,11,20 These CD8 1 cases generally follow an indolent clinical course similar to that of the more common, classic CD4 1 variants. 1,2,5,8,20,21 The CD8 1 immunophenotypic variant of MF, for example, reported by Dummer et al, 21 is characterized by a slowly progressing course, responsiveness to initial treatment with nontoxic, conservative therapy including topical corticosteroids and UVB or PUVA, similar to cases of classic MF. 8,21 Even with recurrence of disease, response to less aggressive skin-directed treatments, including topical chemotherapeutic agents, tends to be very good.…”

Fatal CD8+ Epidermotropic Cytotoxic Primary Cutaneous T-Cell Lymphoma with Multiorgan Involvement

“…Although in our case the latter could not be excluded with certainty, it seems unlikely, based on negative staging performed at another institution at the time of disease onset. Among CTCLs, well-defined types such as MF, pagetoid reticulosis, and CD30+ primary cutaneous large T-cell lymphomas, which may show a CD8+ rather than the classical CD4+ T-cell phenotype, should be ruled out [11,12,13,14]. Indeed, these CD8+ cases have the same clinical behavior and prognosis as the more common CD4+ cases.…”

Characterization of Primary Cutaneous CD8+/CD30+ Lymphoproliferative Disorders

“…[5][6][7][8][9][10][11][12][13][14][15][16][17][18] LyP type D was first described by Saggini et al, 15 demonstrating features of dense infiltrates of medium-sized, pleomorphic epidermotropic lymphocytes in addition to the cytotoxic phenotype. 15 Cutaneous PCALCL with a CD8 + phenotype has also been described, 6,[19][20][21][22][23] which presents a particularly difficult diagnostic challenge since differentiation from primary cutaneous aggressive epidermotropic CD8 + cytotoxic T-cell lymphoma (CTCL) and CD8 + gamma/delta and natural killer/T-cell lymphoma is key to management. 13,14 Furthermore, the term LyP "type E" has also been proposed to describe angiocentric and angiodestructive patterns in LyP.…”