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皮肤淋巴瘤病理会诊的附加值:来自荷兰国家转诊与专业知识中心的两年回顾

Added value of pathology consultations in cutaneous lymphomas: a 2-year review from the Dutch national referral and expertise centre.

作者信息

Doeleman Thom, Beljaards Elise S M, Ottevanger Rosanne, Jansen Patty, Vermeer Maarten H, Quint Koen D, Willemze Rein, Schrader Anne M R

机构信息

Department of Pathology, Leiden University Medical Centre, Leiden, the  Netherlands.

Department of Pathology, University Medical Centre Utrecht, Utrecht, the  Netherlands.

出版信息

Br J Dermatol. 2025 Aug 18;193(3):514-520. doi: 10.1093/bjd/ljaf184.

Abstract

BACKGROUND

The accurate diagnosis of cutaneous lymphomas (CLs) and lymphoproliferative disorders (LPDs) presents significant challenges due to their rarity, the diversity of clinicopathological entities, and the necessity of integrating clinical, immunophenotypic and molecular analyses with histopathology. Misdiagnosis and diagnostic delays are common, potentially leading to inappropriate treatments. The contribution of an expert centre specifically for the pathology of CL has not been previously investigated.

OBJECTIVES

To evaluate the value of pathology consultations at the Dutch national referral centre for CLs focusing on diagnostic agreement and identifying common diagnostic pitfalls.

METHODS

We retrospectively reviewed all pathology consultations concerning CLs received at the Leiden University Medical Centre during 2020 and 2021. Cases were categorized into informative and ambiguous conclusions. Diagnostic agreement between submitting and expert pathologists was assessed and categorized as essential agreement, more specific conclusion, minor disagreement or major disagreement.

RESULTS

A total of 239 consultations from 230 patients were analysed. The submitted conclusion was categorized as informative in 64% of the consultations and as ambiguous in 36%. Most (59%) consultations in our study exhibited essential agreement with the submitting pathologist. The expert centre reduced ambiguous conclusions from 36% to 13%, primarily by resolving differential diagnostic considerations between lymphoma and pseudolymphomatous infiltrates. However, major diagnostic disagreements, with a potentially significant impact on treatment or prognosis, were found in 12% of consultations mostly involving reclassification from benign dermatoses to lymphomas. Mycosis fungoides, primary cutaneous follicle centre lymphoma, CD30+ LPDs and pseudolymphomas were delineated as specific areas of diagnostic difficulty.

CONCLUSIONS

The findings indicate that a national referral and expertise centre for CL pathology contributes to ensuring accurate diagnoses and appropriate patient management. Our data encourage low-threshold consultation in an expert centre for all patients with clinical and/or histological suspicion of CL.

摘要

背景

皮肤淋巴瘤(CLs)和淋巴增殖性疾病(LPDs)的准确诊断面临重大挑战,原因在于其罕见性、临床病理实体的多样性,以及将临床、免疫表型和分子分析与组织病理学相结合的必要性。误诊和诊断延迟很常见,可能导致不适当的治疗。此前尚未对专门针对CL病理学的专家中心的作用进行研究。

目的

评估荷兰国家CL转诊中心病理会诊的价值,重点关注诊断一致性并识别常见的诊断陷阱。

方法

我们回顾性分析了2020年和2021年期间莱顿大学医学中心收到的所有关于CLs的病理会诊。病例分为信息明确和结论模糊两类。评估提交病理学家和专家病理学家之间的诊断一致性,并分为基本一致、更明确的结论、轻微分歧或重大分歧。

结果

共分析了来自230名患者的239次会诊。64%的会诊提交结论被归类为信息明确,36%被归类为结论模糊。我们研究中的大多数(59%)会诊与提交病理学家的意见基本一致。专家中心将结论模糊的比例从36%降至13%,主要是通过解决淋巴瘤与假性淋巴瘤浸润之间的鉴别诊断问题。然而,在12%的会诊中发现了对治疗或预后可能有重大影响的重大诊断分歧,主要涉及从良性皮肤病重新分类为淋巴瘤。蕈样肉芽肿、原发性皮肤滤泡中心淋巴瘤、CD30+LPDs和假性淋巴瘤被确定为诊断困难的特定领域。

结论

研究结果表明,国家CL病理学转诊和专业中心有助于确保准确诊断和适当的患者管理。我们的数据鼓励所有临床和/或组织学怀疑患有CL的患者在专家中心进行低门槛会诊。

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