Multicentric Castleman disease presenting with orbital involvement progressing to TAFRO syndrome: a case report.

BACKGROUND

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.

CASE PRESENTATION

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.

CONCLUSION

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.

SUPPLEMENTARY INFORMATION

The online version contains supplementary material available at 10.1186/s12886-025-04251-0.