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一名年轻西班牙裔女性淋巴结病的罕见病因:隐匿性复发性结节性Rosai-Dorfman病

A Rare Cause of Lymphadenopathy in a Young Hispanic Female: Unmasking Recurrent Nodal Rosai-Dorfman Disease.

作者信息

Alexander Blesset, Alshaikhli Alfarooq, Bartl Mery, Ng-Wong Yilen K, Dulgheru Emilia C

机构信息

Internal Medicine, University of Texas Rio Grande Valley, Edinburg, USA.

Internal Medicine, DHR Health, Edinburg, USA.

出版信息

Cureus. 2023 May 17;15(5):e39131. doi: 10.7759/cureus.39131. eCollection 2023 May.

Abstract

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can present with painless bilateral symmetrical cervical lymphadenopathy, mimicking lymphomas. RDD is characterized by excessive tissue infiltration by dendritic cells, macrophages, or monocyte-derived cells, with a histopathologic diagnosis based on the presence of CD68+, CD163+, and S100+ histiocytes, which differentiate it from other histiocytic neoplasms. In this case report, we present a young Hispanic female with recurrent subcutaneous growths and lymphadenopathy, initially thought to be lymphoma, who was diagnosed with RDD after a significant diagnostic workup. Treatment initially consisted of surgical excision; however, due to recurrence, the patient was successfully treated with corticosteroids and a steroid-sparing agent, 6-mercaptopurine, with significant improvement in symptoms. RDD should be considered a differential diagnosis for patients with cervical lymphadenopathy, and an interdisciplinary approach is essential to managing this rare disorder effectively. The report highlights the need for an interdisciplinary approach to managing this rare disorder effectively and underscores the importance of multimodal treatment in disease suppression. As a rare disease with slow advancement of defined guidelines for diagnostic and treatment strategies, this case report adds to the existing literature on RDD.

摘要

罗萨伊-多夫曼病(RDD)是一种罕见的组织细胞疾病,可表现为无痛性双侧对称性颈部淋巴结病,类似淋巴瘤。RDD的特征是树突状细胞、巨噬细胞或单核细胞衍生细胞过度浸润组织,其组织病理学诊断基于存在CD68 +、CD163 +和S100 +组织细胞,这使其与其他组织细胞肿瘤相区别。在本病例报告中,我们介绍了一名年轻的西班牙裔女性,她反复出现皮下肿物和淋巴结病,最初被认为是淋巴瘤,经过大量诊断检查后被诊断为RDD。治疗最初包括手术切除;然而,由于复发,患者成功接受了皮质类固醇和一种皮质类固醇节省剂6-巯基嘌呤的治疗,症状有显著改善。对于颈部淋巴结病患者,应考虑RDD作为鉴别诊断,采用多学科方法对于有效管理这种罕见疾病至关重要。该报告强调了采用多学科方法有效管理这种罕见疾病的必要性,并强调了多模式治疗在疾病抑制中的重要性。作为一种罕见疾病,其诊断和治疗策略的明确指南进展缓慢,本病例报告为现有的RDD文献增添了内容。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa11/10292128/47ba7eae3244/cureus-0015-00000039131-i01.jpg

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