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伴 T 细胞受体γδ表达的淋巴瘤样丘疹病:26 例未被充分认识的淋巴瘤样丘疹病免疫表型变异型的临床病理病例系列

英文原题:Lymphomatoid Papulosis With T-cell Receptor-Gamma Delta Expression: A Clinicopathologic Case-series of 26 Patients of an Underrecognized Immunophenotypic Variant of Lymphomatoid Papulosis.

PubMed 2024/03/27(内容时间) Am J Surg Pathol Q1 · IF 4.2(JCR 2025)

研究概要

我们从本机构以及通过全面文献综述确定了26例诊断为LyP且具有γδ T细胞的患者,并对这些病例进行了特征分析。

中文摘要

淋巴瘤样丘疹病(LyP)有多种组织病理学表现。以γδ(γδ)T细胞受体表达为特征的LyP可能伪装成侵袭性皮肤T细胞淋巴瘤,尤其是原发性皮肤γδ T细胞淋巴瘤(PCGDTL)或γδ蕈样肉芽肿,并可能被误诊。我们对最大系列的以γδ T细胞表达为特征的LyP进行了临床病理学分析。我们从我们的机构以及通过全面文献复习中识别出26例诊断为伴有γδ T细胞的LyP患者,并对这些病例进行了特征分析。大多数病例接受局部类固醇治疗或未接受任何治疗。大多数病例显示CD4 - CD8 + 表型,并至少表达一种细胞毒性标志物。组织病理学特征包括表皮内或真皮内浸润,伴大细胞和频繁的血管趋向性。1例最初被误诊为PCGDTL,需要进一步治疗。我们的病例系列是国际上最大的以γδ T细胞为主的LyP病例队列,证实了显著临床病理异质性可能导致误诊,并再次强调在处理该鉴别诊断时需要识别典型临床特征、CD30 + T细胞成分和细胞毒性标志物。本研究的局限性包括部分病例的随访、组织学和免疫表型信息较为有限。

展开英文摘要原文

Lymphomatoid papulosis (LyP) has several histopathologic presentations. LyP featuring gamma-delta (γδ) T-cell receptor expression may masquerade as and may be misdiagnosed as aggressive cutaneous T-cell lymphoma, particularly primary cutaneous γδ T-cell lymphoma (PCGDTL) or γδ mycosis fungoides. We performed a clinicopathologic analysis of the largest series of LyP featuring γδ T-cell expression. We identified 26 patients with a diagnosis of LyP with γδ T cells from our institutions, as well as through a comprehensive review of the literature, and characterized these cases. Most cases were treated with topical steroids or not treated at all. The majority of cases showed a CD4 - CD8 + phenotype and featured at least one cytotoxic marker. Histopathologic features included an intraepidermal or dermal infiltrate with large cells and frequent angiotropism. One case was initially misdiagnosed as PCGDTL, requiring further therapy. Our case series, the largest international cohort of γδ T cell predominant LyP cases, confirms marked clinicopathologic heterogeneity that may contribute to misdiagnosis, reasserting the need to identify classic clinical features, CD30 + T-cell components, and markers of cytotoxicity when dealing with this differential diagnosis. A limitation of this study includes somewhat limited follow-up, histologic, and immunophenotypic information for some cases.

论文信息

作者
Mark E、Kempf W、Guitart J、Pulitzer M、Mitteldorf C、Hristov A、Torres-Cabala C、Marchi E
单位
Department of Pathology.
文献类型
综述
期刊
The American journal of surgical pathology2024 May 1
原文标识
PubMed 38533681 · DOI 10.1097/PAS.0000000000002200