Iris lymphoma in a pediatric cardiac transplant recipient: Clinicopathologic findings

Purpose: To report the unusual presentation of a confirmed iris lymphoma in the setting of posttransplant lymphoproliferative disorder. Design: Case report with clinicopathologic findings. Methods: The medical record was reviewed for clinical manifestations, course of disease, and pathologic and radiologic findings. Results: A 4-year-old girl who had undergone a cardiac transplant at 3 months of age was found to have unilateral iris lesions associated with granulomatous uveitis. Her condition was unresponsive to local and systemic steroids, so she underwent an iris biopsy that was found to contain a B cell-derived monoclonal lymphoproliferation. Treatment with systemic chemotherapy including cyclophosphamide and prednisone resulted in a transient, partial response. Localized radiotherapy induced total regression of the iris lesions. She continues to be observed and has been free of recurrence or metastasis for the past 12 months. Conclusions: Because of the increased use of organ transplantation, eye care specialists should consider the diagnosis of intraocular lymphoma when confronted with uveitis-like syndrome unresponsive to topical steroid therapy. Although there are numerous treatment options for intraocular lymphoma, radiation therapy was found to be effective in the treatment of this case. (C) 2000 by the American Academy of Ophthalmology.