Indolent CD8+ lymphoid proliferation of the ear: A phenotypic variant of the small‐medium pleomorphic cutaneous T‐cell lymphoma?

Abstract

Our observation confirms that some patients present with a peculiar lymphoid proliferation of small-medium pleomorphic cytotoxic lymphocytes located on the ear, probably representing a phenotypic variant of the cutaneous small/medium pleomorphic T-cell lymphoma (CSMPTCL). These cases should not be misinterpreted as a high-grade cytotoxic lymphoma.

Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature

“…14 There is speculation that these indolent CD8 1 lymphoid proliferations and PCSM-TCL represent phenotypic variants of a single condition, as both entities share an apparent indolent nature, similar anatomic locations, and common histopathologic features. 13 More definitive conclusions regarding the appropriate categorization of these entities may be facilitated by molecular analysis and long-term follow-up.…”

Indolent T-cell lymphoproliferative disease of the gastrointestinal tract

“…[19][20][21][22] These proliferations most commonly present as nodules on the external ear, are more common in men, and persist if left untreated but do not progress to aggressive lymphoma. By immunohistochemistry, these lesions are also positive for CD8 and TCR-BF1 and express the cytotoxic marker TIA1.…”

“…19,21,22 Most are clonal by TCR-g chain gene rearrangement. [19][20][21][22] A possible relationship of this indolent CD8-positive lymphoid proliferation of the face with indolent T-LPD of the GI tract is uncertain because the etiology of both conditions is unknown.…”

Early lymphoid lesions: conceptual, diagnostic and clinical challenges

“…T-cell clonality has been demonstrated in most cases. 90,91 Initial evidence supports an indolent behavior with localized disease, and good response to surgery and radiotherapy. Rare patients have had persistent disease or local relapse but no deaths have been reported.…”

“…Indolent CD8-positive lymphoid proliferation of the skin was described initially as a solitary nodule of the ear 90,91 but is now known to occur at other anatomic sites, 92 including the distal extremities. 93 Histology typically shows a non-epidermotropic, dense dermal lymphoid infiltrate composed of medium-sized atypical CD8 + T cells positive for TIA-1, but negative for Granzyme B ( Figure 3D-F).…”