Ali Liaqat, Young Michelle R, Bayerl Michael G, Helm Klaus F, Clarke Loren E
Department of Pathology, The Penn State Hershey Medical Center, Hershey, PA, USA.
Department of Dermatology, The Penn State Hershey Medical Center, Hershey, PA, USA.
J Cutan Pathol. 2015 Dec;42(12):987-991. doi: 10.1111/cup.12591. Epub 2015 Oct 19.
The precise classification and characterization of primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) has been hindered by clinical and morphologic features that overlap with other lymphomas, especially subcutaneous panniculitis-like T cell lymphoma (SPTCL). The recent World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification distinguishes the more aggressive PCGD-TCL from the usually indolent SPTCL, however. We report a 30-year-old woman with an indurated violaceous plaque on the left cheek that had been present for several years. Biopsies showed a dense lymphocytic infiltrate involving the subcutis and dermis that consisted mostly of small and medium-sized lymphocytes, some with irregular nuclear contours and dense chromatin. These cells were positive for TIA-1, TCR-gamma and CD8, but negative for beta-F1 and granzyme-B. Staging with positron emission tomography-computed tomography (PET/CT), CBC and bone marrow with flow cytometry identified lymphadenopathy as well as blood and marrow involvement by an abnormal TCRgd-positive T-cell proliferation (Ann Arbor Stage IV). The patient's history of a long-standing lesion in this case is unusual, in that gamma-delta T-cell lymphomas are typically rapidly progressive neoplasms. As such, it raises the possibility of 'transformation' of a long-standing inflammatory process into an overt lymphoma.
原发性皮肤γ-δ T细胞淋巴瘤(PCGD-TCL)的精确分类和特征描述一直受到与其他淋巴瘤重叠的临床和形态学特征的阻碍,尤其是皮下脂膜炎样T细胞淋巴瘤(SPTCL)。然而,最近的世界卫生组织/欧洲癌症研究与治疗组织(WHO/EORTC)分类将侵袭性更强的PCGD-TCL与通常惰性的SPTCL区分开来。我们报告一名30岁女性,左脸颊有一个硬结性紫斑,已存在数年。活检显示皮下组织和真皮有密集的淋巴细胞浸润,主要由中小淋巴细胞组成,部分细胞核轮廓不规则,染色质致密。这些细胞TIA-1、TCR-γ和CD8呈阳性,但β-F1和颗粒酶B呈阴性。通过正电子发射断层扫描-计算机断层扫描(PET/CT)、全血细胞计数(CBC)以及骨髓流式细胞术进行分期,发现存在淋巴结病以及血液和骨髓受累,有异常的TCRgd阳性T细胞增殖(Ann Arbor分期IV期)。该患者长期病变的病史并不常见,因为γ-δ T细胞淋巴瘤通常是快速进展的肿瘤。因此,这增加了长期炎症过程“转变”为明显淋巴瘤的可能性。