Cone damage in patients receiving high-dose irofulven treatment

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 Muller 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.