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两例具有γ/δ+表型且病程惰性的原发性皮肤淋巴瘤:皮肤γ/δ+ T细胞淋巴瘤异质性的进一步证据

Two cases of primary cutaneous lymphoma with a γ/δ+ phenotype and an indolent course: further evidence of heterogeneity of cutaneous γ/δ+ T-cell lymphomas.

作者信息

Kempf Werner, Kazakov Dmitry V, Scheidegger Paul E, Schlaak Max, Tantcheva-Poor Iliana

机构信息

*Kempf Und Pfaltz Histologische Diagnostik, Zürich, Switzerland; †Department of Pathology, Faculty of Medicine in Pilsen, Charles University in Prague, Prague, Czech Republic; ‡Dermatology Practice, Brugg, Switzerland; and §Department of Dermatology, University of Cologne, Cologne, Germany.

出版信息

Am J Dermatopathol. 2014 Jul;36(7):570-7. doi: 10.1097/DAD.0000000000000029.

Abstract

Cutaneous γ/δ+ 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 γ/δ+ phenotype by immunohistochemistry. We report 2 patients with epidermotropic cutaneous T-cell lymphomas displaying a γ/δ+ 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 γ/δ+ 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.

摘要

皮肤γ/δ+ T细胞淋巴瘤(CGD-TCL)是一种罕见但侵袭性强的淋巴瘤,大多数患者预后较差。其临床病理谱具有多样性,主要包括呈斑片和斑块表现的亲表皮浸润,或伴有真皮和/或皮下浸润的肿瘤。CGD-TCL的诊断需要通过免疫组织化学证实γ/δ+表型。我们报告2例表现为γ/δ+表型的亲表皮性皮肤T细胞淋巴瘤患者,但病程呈惰性。1例患者的临床表现类似于斑块期蕈样肉芽肿,但病变内反复出现水疱形成,并伴有发热和关节痛,而另1例患者左侧颞部出现2处局限性糜烂性斑块,有密集的亲表皮性及真皮弥漫性和毛囊性非典型中小淋巴细胞浸润。这些病例证实了以下观点:皮肤T细胞淋巴瘤中γ/δ+表型的表达本身并不预示预后更差,且CGD-TCL可能代表一个临床和预后异质性的群体。

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