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皮肤 T 细胞淋巴瘤伴皮肤外转移的临床病理特征:病例系列研究。

Clinicopathologic Features of Cutaneous T-Cell Lymphomas With Extracutaneous Metastasis: A Case Series.

机构信息

Department of Pathology, Yale School of Medicine, New Haven, CT.

Department of Pathology, Brigham and Women's Hospital, Boston, MA.

出版信息

Clin Lymphoma Myeloma Leuk. 2023 Nov;23(11):e405-e410. doi: 10.1016/j.clml.2023.08.006. Epub 2023 Aug 14.

Abstract

BACKGROUND

In advanced stages, Cutaneous T-cell lymphomas (CTCL) can metastasize to extracutaneous regions. CTCL with metastasis exhibits unique clinicopathologic characteristics.

PATIENTS AND METHODS

This study collected 35 cases of primary CTCL with extracutaneous metastasis from a single institution over a period of 20 years. Clinicopathologic features including demographics, CD30 expression, large cell transformation, metastatic sites, T-cell receptor clonality studies and survival data were analyzed.

RESULTS

The study identified various CTCL entities including mycosis fungoides (MF), Sezary syndrome (SS), cutaneous anaplastic large cell lymphoma (C-ALCL), and primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS). Limited data showed that metastasis can be independent of large cell transformation and/or CD30 expression. Lymph nodes were the most common site of metastasis, followed by the bone marrow. Oropharyngeal metastasis is likely to accompany visceral organ or brain metastasis (P = .049). MF had a longer interval to metastasis than SS (P = .038). Patients with lymph node only metastasis have better survival than patients with metastasis to other sites (P = .012).

CONCLUSION

To the best of our knowledge, there are limited studies analyzing the clinicopathologic features of different CTCL entities with metastasis as a single population. This research provides valuable insights into the unique characteristics of metastatic CTCL.

摘要

背景

在晚期,皮肤 T 细胞淋巴瘤(CTCL)可能转移到皮肤外区域。转移性 CTCL 表现出独特的临床病理特征。

患者和方法

本研究从一家机构收集了 20 年来的 35 例原发性 CTCL 伴皮肤外转移病例。分析了临床病理特征,包括人口统计学、CD30 表达、大细胞转化、转移部位、T 细胞受体克隆性研究和生存数据。

结果

研究确定了各种 CTCL 实体,包括蕈样真菌病(MF)、Sezary 综合征(SS)、皮肤间变大细胞淋巴瘤(C-ALCL)和原发性皮肤外周 T 细胞淋巴瘤,非特指型(pcPTCL-NOS)。有限的数据表明,转移可以独立于大细胞转化和/或 CD30 表达。淋巴结是最常见的转移部位,其次是骨髓。口咽转移可能伴有内脏器官或脑转移(P=0.049)。MF 转移到其他部位的间隔时间长于 SS(P=0.038)。只有淋巴结转移的患者比有其他部位转移的患者生存更好(P=0.012)。

结论

据我们所知,将具有转移的不同 CTCL 实体作为单一人群进行分析的临床病理特征研究有限。本研究提供了关于转移性 CTCL 独特特征的有价值的见解。

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