Wei Yuanfeng, Qi Jing, Yan Jiawei, Gao Guangjian, Ji Xinyue, Yu Danning, Chen Wanru, Huang Dongping
Department of Hematology, The First Affiliated Hospital of Wannan Medical College, No.2, West Zheshan Road, Wuhu, People's Republic of China.
Department of Imaging, The First Affiliated Yijishan Hospital of Wannan Medical College, Wuhu, People's Republic of China.
BMC Ophthalmol. 2025 Jul 22;25(1):421. doi: 10.1186/s12886-025-04251-0.
Castleman Disease (CD) can be unicentric or multicentric, with the ocular involvement rare; notably, the aggressive TAFRO syndrome (TS) is a subtype of idiopathic multicentric CD (iMCD). Ocular involvement in CD is rare, and to date, there are no reported cases of progression from ocular involvement to TAFRO syndrome.
A 45-year-old woman with a six-year history of progressive, non-painful eyelid redness and protrusion presented with recent systemic symptoms including limb edema and fever. Laboratory tests indicated anemia, thrombocytopenia, and elevated levels of Interleukin (IL)-6 and vascular endothelial growth factor (VEGF), while imaging revealed bilateral orbital masses, multiple lymphadenopathies, splenomegaly, and pleural and abdominal effusions. Diagnosed with iMCD-TAFRO syndrome through comprehensive laboratory tests and multi-site biopsies. After intensive chemotherapy treatment, the patient achieved complete remission (CR) and is currently under regular follow-up.
Here, we present the first reported case of iMCD initially manifesting with ocular symptoms, subsequently progressing to TS, highlighting the need for heightened clinical awareness and multidisciplinary collaboration in the diagnosis and management of such atypical aggressive disease.
The online version contains supplementary material available at 10.1186/s12886-025-04251-0.
Castleman病（CD）可分为单中心型或多中心型，眼部受累罕见；值得注意的是，侵袭性TAFRO综合征（TS）是特发性多中心CD（iMCD）的一种亚型。CD的眼部受累罕见，迄今为止，尚无从眼部受累进展为TAFRO综合征的病例报道。
一名45岁女性，有6年进行性、无痛性眼睑发红和突出病史，近期出现包括肢体水肿和发热在内的全身症状。实验室检查显示贫血、血小板减少，白细胞介素（IL）-6和血管内皮生长因子（VEGF）水平升高，影像学检查发现双侧眼眶肿块、多处淋巴结肿大、脾肿大以及胸腔和腹腔积液。通过全面的实验室检查和多部位活检诊断为iMCD-TAFRO综合征。经过强化化疗治疗，患者实现完全缓解（CR），目前正在定期随访中。
在此，我们报告首例最初表现为眼部症状，随后进展为TS的iMCD病例，强调在诊断和管理这种非典型侵袭性疾病时需要提高临床意识并进行多学科协作。
在线版本包含可在10.1186/s12886-025-04251-0获取的补充材料。
