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Michael S. Lee, MD; Nisha Gupta, PhD; Richard T. Penson, MD, MRCP; John Loewenstein, MD; Meredith S. Wepner; Michael V. Seiden, MD, PhD; Ann H. Milam, PhD

Arch Ophthalmol. 2005;123:29-34.

Objectives  To describe the clinical, perimetric, and electroretinographic (ERG) results of 4 patients with cone dysfunction following irofulven treatment including the histopathologic and immunocytochemical features of one patient’s retinas.

Design  Observational case series.

Methods  The patients were examined clinically, including perimetric and ERG evaluations. Eyes from patient 1 and healthy postmortem eyes were processed for histopathologic and immunocytochemistry studies with antibodies specific for cones, rods, and reactive Müller cells.

Main Outcome Measures  Clinical signs and symptoms, perimetry, ERG, retinal histopathologic and immunocytochemistry study results.

Results  All 4 patients had ERG changes consistent with abnormal cone responses and relatively normal rod responses. Compared with control eyes, the retina of patient 1 had approximately half the normal numbers of macular cones and fewer peripheral cones. The number of rods were normal but all rod and cone outer segments were shortened.

Conclusion  High-dose irofulven treatment causes cone-specific damage with relative sparing of rods.

Author Affiliations: Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio (Dr Lee); the M. Kirby Center for Molecular Ophthalmology and the Scheie Eye Institute, University of Pennsylvania, Philadelphia (Drs Gupta and Milam and Ms Wepner); Division of Hematology-Oncology, Massachusetts General Hospital, Boston (Drs Penson and Seiden); and the Retina Service, Massachusetts Eye and Ear Infirmary, Boston (Dr Loewenstein).

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