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Two cases of primary cutaneous lymphoma with a γ/δ+ phenotype and an indolent course


Kempf, Werner; Kazakov, Dmitry V; Scheidegger, Paul E; Schlaak, Max; Tantcheva-Poor, Iliana (2014). Two cases of primary cutaneous lymphoma with a γ/δ+ phenotype and an indolent course. American Journal of Dermatopathology, 36(7):570-577.

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. These cases corroborate the view that expression of a [gamma]/[delta]+ 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.

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. These cases corroborate the view that expression of a [gamma]/[delta]+ 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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2014
Deposited On:13 Feb 2015 13:30
Last Modified:05 Apr 2016 18:54
Publisher:Lippincott Williams & Wilkins
ISSN:0193-1091
Publisher DOI:https://doi.org/10.1097/DAD.0000000000000029
Permanent URL: https://doi.org/10.5167/uzh-106108

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