Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Purpose: The purpose of this study was to report a case of bilateral blindn ess, bilateral necrotizing scleritis, and bilateral deafness in a patient w ith myelodysplastic syndrome (MDS), In such a patient, the possibility of r elapsing polychondritis (RPC) associated with MDS must be considered. Case Report/Methods: A 66-year-old patient suffered from myelodysplastic sy ndrome (MDS), Shortly before his death, he became bilaterally blind and dea f. A biopsy was taken from the conjunctiva and the bone marrow, and both ey es were obtained after death for further investigation. Findings of the cli nical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. Results: Ocular sections showed a diffuse necrotizing scleritis with modera te uveitis and no identifiable infectious agent. Neither was there any evid ence of a leukemic infiltration. - The deafness had been due to inner ear f ailure, and the patient died of a cardiac failure. Conclusions: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC), Furthermore, RPC ca n be associated with MDS, Thus, in addition to leukemic infiltration and in fection involving ocular structures ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential c omplications.