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

IF 1.6 4区 医学 Q3 DERMATOLOGY
Craig M. Fisher MD, Steven F. Capen MD, Justin P. Bandino MD, Allen R. Holmes MD, Christine M. Lee MD
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引用次数: 0

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.

原发性皮肤γ/δ(γδ)T细胞淋巴瘤的明显组织嗜血细胞淋巴组织细胞增多症。
原发性皮肤γ/δ(γδ)T细胞淋巴瘤(PCGDTCL)是一种罕见的侵袭性恶性肿瘤,由皮肤和皮下的γδT淋巴细胞组成。我们报告了一例具有挑战性的 PCGDTCL 病例,患者是一名 35 岁的男性士兵,在被部署到伊拉克和叙利亚后出现了全身症状、泛发性淋巴细胞增多症、嗜血细胞淋巴组织细胞增多症(HLH)、播散性淋巴结病以及四肢和背部的皮肤病变。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,并被周围组织中主要的HLH所掩盖。病理学家应该认识到,PCGDTCL 的诊断可能会受到 HLH 的影响,因此需要进行多次活检和全面的免疫组化检查。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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