Ramani Nisha S, Curry Jonathan L, Merrill E Dean, Aung Phyu P, Prieto Victor, Tetzlaff Michael T, Duvic Madeleine, Miranda Roberto N, Torres-Cabala Carlos
*Department of Pathology, SUNY Upstate Medical University, Syracuse, NY; †Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, TX; Departments of ‡Dermatology, and §Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX.
Am J Dermatopathol. 2016 Oct;38(10):e147-9. doi: 10.1097/DAD.0000000000000608.
Primary cutaneous γ/δ T-cell lymphoma (PCGDTCL) accounts for <1% of all primary cutaneous lymphomas. These rare diseases are believed to originate from γ/δ lymphocytes. Clinical presentation may vary, but its clinical behavior is regarded as aggressive and long-term survival is anecdotal. This study describes the case of a 60-year-old man with multiple, rapidly progressing skin plaques on his head, arms, torso, buttocks, and legs. The histopathological changes seen in the skin biopsy were extraordinarily subtle with mild epidermal hyperplasia and a very sparse lymphoid infiltrate involving epidermis and superficial dermis. Immunohistochemical studies revealed the atypical intraepidermal hyperchromatic cells to be mostly positive for CD3 and CD7 and negative for both CD4 and CD8. The intraepidermal atypical lymphocytes were positive for TCR gamma, and negative for betaF1 and CD56. The clinical, morphologic, and immunohistochemical findings supported the diagnosis of PCGDTCL. This case illustrates a case of epidermotropic variant of PCGDTCL that, albeit a bland histopathological presentation, was associated with an aggressive clinical behavior.
原发性皮肤γ/δ T细胞淋巴瘤(PCGDTCL)占所有原发性皮肤淋巴瘤的比例不到1%。这些罕见疾病被认为起源于γ/δ淋巴细胞。临床表现可能各不相同,但其临床行为被视为侵袭性的,长期生存的情况鲜有报道。本研究描述了一名60岁男性的病例,其头部、手臂、躯干、臀部和腿部出现多个迅速进展的皮肤斑块。皮肤活检中所见的组织病理学变化非常细微,表现为轻度表皮增生以及累及表皮和浅表真皮的非常稀疏的淋巴细胞浸润。免疫组织化学研究显示,表皮内非典型的嗜染细胞大多CD3和CD7呈阳性,CD4和CD8均为阴性。表皮内非典型淋巴细胞TCRγ呈阳性,βF1和CD56呈阴性。临床、形态学和免疫组织化学检查结果支持PCGDTCL的诊断。该病例说明了一例PCGDTCL的亲表皮性变体,尽管其组织病理学表现不明显,但与侵袭性临床行为相关。