EBV-induced polymorphic lymphoproliferative disorder of the iris after heart transplantation

Abstract

Background: Posttransplantation lymphoproliferative disorder (PTLD) of the iris is a rare entity with only ten cases having been published as yet. Its clinical aspect is typical. Therapy is multimodal and affords an interdisciplinary approach.

Methods: A 7-year-old boy developed a lymphoproliferative mass of the iris with uveitis 4 years after heart transplantation and immunosuppression. A progressive, flesh-colored thickening of the iris with secondary angle closure glaucoma necessitated a diagnostic and therapeutic iridectomy. Morphological investigation of the iris specimen disclosed a polymorphic posttransplantation lymphoproliferative disorder (PTLD) and the presence of Epstein-Barr virus (EBV) within the tissue. The EBV load in peripheral blood monocytes was massively elevated, thus indicating a chronic EBV infection. After conservative treatment and radiation therapy, the iris mass quickly resolved. There was no evidence of systemic PTLD.

Conclusions: PTLD is a well-known, EBV-induced entity that rarely affects the eye. EBV is principally detectable in specimens of iris PTLD. If conservative, antiviral treatment fails, the iris lesions can be treated by local radiation therapy with very good success. In the near future, patients with PTLD of the eye may benefit from immunologic treatment with ex vivo generation of virus-specific T-lymphocytes.