Ocular Involvement of Relapsed Nodal T Follicular Helper Cell Lymphoma, Angioimmunoblastic Type

A 70-year-old woman diagnosed with nodal T follicular helper cell lymphoma, angioimmunoblastic type (nTFHL-AI), was treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). Approximately 2 weeks after the fifth cycle of CHOP, the patient developed difficulty in keeping her right eye open. Simultaneously, cervical lymphadenopathy was observed. Eye examination revealed solid subconjunctival tissue, chemosis, conjunctival hyperemia, eyelid redness/swelling, and diplopia (Figure A,B). Magnetic resonance imaging revealed contrast enhancement in the soft tissue surrounding the right eyeball (Figure C). Positron emission tomography revealed hypermetabolic masses on the right eyelid and eyeball (Figure D). Conjunctival biopsy was performed, and tissue samples were obtained from the inferonasal and inferotemporal conjunctiva. Hematoxylin and eosin-stained sections demonstrated the infiltration of small- to medium-sized lymphoid cells into the lamina propria (Figure E). Immunohistochemical analysis revealed that the lymphoid cells were positive for CD3, CD4, CD5, BCL-6, PD-1, ICOS, and CXCL-13 (Figure F–H) and negative for CD8, CD20, CD30, and EBER, which were matched as the IHC profile of nTFHL-AI. Based on these findings, the conjunctival lesion was diagnosed as an nTFHL-AI relapse. Cervical lymph node biopsy also confirmed nTFHL-AI relapse and identified clonal T-cell receptor-β gene rearrangement. The patient received salvage chemotherapy with ifosfamide, carboplatin, and etoposide, which proved ineffective. In contrast, the patient responded to involved-site radiation therapy of the right eye combined with tucidinostat, an oral histone deacetylase inhibitor. Ocular adnexal lymphoma accounts for 1%–2% of all non-Hodgkin lymphomas, most of which are of the B-cell lineage [1]. Conjunctival T-cell lymphoma is rare, with conjunctival nTFHL-AI being extremely uncommon [2, 3]. Conjunctival lymphomas exhibit distinct ocular features by subtype and are particularly useful for diagnosing common B-cell lymphomas [2]. Conversely, ocular manifestations are often insufficiently described in T-cell lymphomas, which are rare subtypes of conjunctival lymphomas [3-7]. In a previously reported case of nTFHL-AI, the ocular findings comprised two pink, discrete, non-tender bulbar conjunctival masses [3]. In our case, in addition to a solid conjunctival mass, we observed chemosis, conjunctival hyperemia, eyelid redness and swelling, and diplopia. Our case presents a rare manifestation of ocular involvement of nTFHL-AI and provides insights into the differential diagnosis of a red eye, which includes both benign and serious conditions requiring ophthalmologic evaluation, including infections, rheumatologic disorders, thyroid diseases, sarcoidosis, orbital myositis, and, rarely, malignancies. The authors have nothing to report. The authors have nothing to report. The authors have nothing to report. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. The authors declare no conflicts of interest. Data from this study are not deposited in a publicly accessible database.