Orthopaedic Department, Sarawak General Hospital, Kuching, Malaysia.
Orthopaedic Department, Hospital Queen Elizabeth, Kota Kinabalu, Malaysia.
BMJ Case Rep. 2022 Mar 23;15(3):e245360. doi: 10.1136/bcr-2021-245360.
Classic Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with bilateral massive painless cervical lymphadenopathy. It is a mysterious disease and there is little knowledge of its pathogenesis, clinical features, radiological findings, laboratory investigations, effective treatments and prognosis. Some of its clinical presentations may overlap with those of infection. Just like tuberculosis infection, RDD may involve many other organs, for example, skin, kidney, bone, brain and spine. The diagnosis can easily be overlooked, especially in communities hyperendemic to tuberculosis infection. We report our experience in diagnosing and managing a patient with spinal RDD with concurrent tuberculosis infection, who was treated empirically for cervical tuberculous lymphadenitis without a conclusive laboratory finding prior to her spinal condition. In view of her acute neurological deficit, emergency spinal decompression was performed. Her intraoperative spinal samples had shown classic histopathological features of RDD. We believe the lymphadenopathy was part of the clinical presentation of RDD. She showed favourable neurological recovery throughout the follow-up.
经典 Rosai-Dorfman-Destombes 病（RDD）是一种罕见的组织细胞增生症，其特征为双侧无痛性巨大颈淋巴结病。该病的发病机制、临床特征、影像学表现、实验室检查、有效治疗和预后均不明确，部分临床表现可能与感染性疾病重叠。与结核感染一样，RDD 可累及多个器官，如皮肤、肾脏、骨骼、大脑和脊柱等。由于该病在结核感染高发地区易被忽视，诊断较为困难。本文报道了 1 例脊柱 RDD 合并结核感染患者的诊治经验，该患者颈椎结核性淋巴结炎在脊柱病变前曾经验性治疗，无明确的实验室检查结果。鉴于患者存在急性神经功能缺损，行紧急脊柱减压术。术中脊柱标本的组织病理学表现具有典型的 RDD 特征。我们认为，淋巴结病是 RDD 临床表现的一部分。患者在随访过程中神经功能恢复良好。
