A novel presentation of ocular chronic lymphocytic leukemia.

[PURPOSE] To describe a rare presentation of chronic lymphocytic leukemia (CLL) involving the conjunctiva and caruncle.

[OBSERVATIONS] A 64-year-old male with a history of systemic CLL and a blind right eye due to retinal vascular disease and optic neuropathy presented with gradually worsening redness and foreign body sensation in the same eye. Anterior exam revealed chemosis, thickened conjunctiva, an enlarged caruncle with an irregular surface, and large pale superior and inferior forniceal follicles, altogether concerning for infiltration of the conjunctiva by CLL. Conjunctival and caruncular biopsies demonstrated small CD5 B-cell lymphoma, consistent with CLL.

[CONCLUSIONS AND IMPORTANCE] CLL is the most common form of leukemia in the western world, though there is a relatively low prevalence of ocular manifestations. Our case revealed a unique presentation of this disease manifesting in the conjunctiva and caruncle. Performing an incisional biopsy of the abnormal appearing tissue for analysis with cell typing was essential to confirming this diagnosis. This case reveals a novel presentation of CLL involving the caruncle and emphasizes the importance of obtaining a tissue biopsy in patients presenting with atypical changes seen on the conjunctiva and caruncle with a history of known hematologic malignancy.