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显著的组织噬血细胞性淋巴组织细胞增生症掩盖了原发性皮肤 γ/δ(γδ)T 细胞淋巴瘤。

Prominent tissue hemophagocytic lymphohistiocytosis obscuring primary cutaneous gamma/delta (γδ) T-cell lymphoma.

机构信息

Department of Dermatology, Wilford Hall Ambulatory Surgical Center, JBSA Lackland, Texas, USA.

Department of Pathology and Area Laboratory Services, Brooke Army Medical Center, JBSA-Fort Sam Houston, Texas, USA.

出版信息

J Cutan Pathol. 2024 Dec;51(12):959-963. doi: 10.1111/cup.14718. Epub 2024 Sep 18.

DOI:10.1111/cup.14718
PMID:39295229
Abstract

Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive malignant neoplasm of γδ T lymphocytes arising within the skin and subcutis. We present a challenging case of PCGDTCL diagnosed in a 35-year-old male soldier who presented with constitutional symptoms, pancytopenia, hemophagocytic lymphohistiocytosis (HLH), disseminated lymphadenopathy, and cutaneous lesions on his extremities and back following a deployment to Iraq and Syria. Histopathologic evaluation of an excisional biopsy showed that PCGDTCL can be focal, localized to the subcutaneous adipose tissue, and obscured by predominant HLH in the surrounding tissues. Pathologists should recognize that the diagnosis of PCGDTCL may be confounded by florid HLH and require multiple biopsies and a comprehensive immunohistochemical panel.

摘要

原发性皮肤 γ/δ(γδ)T 细胞淋巴瘤(PCGDTCL)是一种罕见的、侵袭性的恶性肿瘤,由皮肤和皮下组织内的 γδ T 淋巴细胞引起。我们报告了一例 35 岁男性士兵的挑战性病例,他在部署到伊拉克和叙利亚后出现全身症状、全血细胞减少、噬血细胞性淋巴组织细胞增生症(HLH)、全身性淋巴结病和四肢及背部皮肤病变,被诊断为 PCGDTCL。切除活检的组织病理学评估显示,PCGDTCL 可能是局灶性的,局限于皮下脂肪组织,并被周围组织中占主导地位的 HLH所掩盖。病理学家应该认识到,PCGDTCL 的诊断可能因明显的 HLH而变得复杂,需要多次活检和全面的免疫组织化学分析。

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