Two Cases of Primary Cutaneous Lymphoma With a γ/δ+ Phenotype and an Indolent Course

Abstract

Cutaneous [gamma]/[delta]+ T-cell lymphoma (CGD-TCL) is a rare but aggressive lymphoma associated with a poor prognosis in most patients. The clinicopathological spectrum is variable including predominantly epidermotropic infiltrates manifesting with patches and plaques or tumors with dermal and/or subcutaneous infiltrates. The diagnosis of CGD-TCL requires the demonstration of a [gamma]/[delta]+ phenotype by immunohistochemistry. We report 2 patients with epidermotropic cutaneous T-cell lymphomas displaying a [gamma]/[delta]+ phenotype, but exhibiting an indolent course. In one patient, the clinical presentation was similar to mycosis fungoides in patch and plaque stage, but recurrent blister formation within the lesions was observed accompanied by fever and arthralgias, whereas the second patient presented with 2 localized erosive plaques on the left temple and dense epidermotropic and dermal diffuse and folliculotropic infiltrates of atypical small-to-medium-sized lymphocytes. exhibiting an indolent course. In one patient, the clinical presentation was similar to mycosis fungoides in patch and plaque stage, but recurrent blister formation within the lesions was observed accompanied by fever and arthralgias, whereas the second patient presented with 2 localized erosive plaques on the left temple and dense epidermotropic and dermal diffuse and folliculotropic infiltrates of atypical small-tomedium-sized lymphocytes. These cases corroborate the view that expression of a g/d+ phenotype in cutaneous T-cell lymphomas per se does not portend a worse prognosis and that CGD-TCL may represent a clinically and prognostically heterogeneous group.

Primary cutaneous γδ T‐cell lymphoma with unusual immunophenotype: A case report and review of published work

“…While the prognosis of CGD‐TCL is generally poor, indolent CGD‐TCL cases have also been reported . Particularly, MF‐like cases tended to have a good prognosis.…”

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

“…However, recent studies have identified numerous cases with epidermotropism. 10,11 In our meta-analysis, we found that nearly half of patients had some degree of epidermotropism in at least one of their biopsy specimens, and nearly a quarter presented with disease that was predominantly epidermotropic. For pathologists, this may dramatically shift the paradigm of the differential diagnosis of an epidermotropic T-cell infiltrate.…”

“…However, due to the publication of several series examining the survival of patients with epidermotropic disease in selected patients, this population may be enriched in our data set. This may be a strength of our report: given that several publications have collected epidermotropic cases with good survival outcomes, 10,11 our population should be enriched for patients with such patients, and we still do not detect a survival benefit to this phenotype. Rare clinical presentations (eg, eczema-like or ulcerated) may also be enriched in this population, although the overall trend of patients presenting with nodules or tumors with or without ulceration does hold true to previously published literature.…”

“…Evidence, in the form of case serieses, has been provided in the literature that patients with epidermotropic PCGDTCL may have an improved prognosis compared with those with only dermal and/or subcutaneous involvement. 10,11 In this article, we seek to achieve three goals: (a) identify the incidence of epidermotropism in the PCGDTCL literature, (b) assess the survival benefit potentially associated with epidermotropism or predominantly epidermotropic phenotype, and (c) compare immunophenotype between PCGDTCL patients with and without epidermotropism. This work also aims to highlight this under-recognized histopathologic type as a means to help improve recognition and diagnosis.…”

Primary Cutaneous Gamma-Delta T-cell Lymphoma: Not an Aggressive Disease in All Cases

“…In this particular subset, regardless of its likely heterogeneity, clinical presentation appears to be similar to the main, aggressive subset with nodules, indurated plaques, ulcers or blistering lesions ( 11 ) located mainly on the limbs and trunk but which may also affect the face ( 5 , 11 ) or display a widespread distribution. Interestingly Merrill et al ( 13 ) suggested that a predominantly epidermotropic pattern might be associated with a more favourable outcome, although most other reported cases of “indolent” PCGDTCL displayed a more usual predominant dermal and/or subcutaneous involvement as in our 2 patients, even though a sparse epidermotropism was occasionally observed in patient 2.…”