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S. Barile, G. Cordahi, R. Thibeault, Y. Robitaille; Post Transplantation Lymphoproliferative Disorder With Chorioretinal Involvement After Bone Marrow Transplant in a Child. Invest. Ophthalmol. Vis. Sci. 2007;48(13):3679. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Epstein-Barr (EBV) associated post transplantation lymphoproliferative disorder (PTLD) has emerged as an increasing cause of high morbidity and mortality in solid organ and bone marrow transplant recipients, particularly in children. Clinical presentation is highly variable, however, chorioretinal involvement has been rarely described. We report a case of confirmed chorioretinal PTLD, CD20 negative, induced by EBV after hematological stem cell transplantation (HSCT) in a child and its response to radiation treatment.
Seven months post HSCT a bilateral anterior non granulomatous uveitis associated with hemorrhagic chorioretinitis and a retinal detachment developed in a 8 year old girl. Serology was positive for CMV and EBV. Vitreous and retinal biopsies were performed during pars plana vitrectomy and quantitative PCR for EBV was realised on the vitreous fluid.
EBV-PCR on the vitreous sample revealed high viral load although it was negative in blood. The retinal biopsy showed a complete retinochoroidal infiltration by lymphoproliferative cells LMP-1 positive, but negative for CD20. In situ hybridization with Eber probe confirmed the presence of EBV. Treatment consisted of local radiation therapy (150 Gy). Five months later vision stabilized and chorioretinal infiltrates healed in both eyes without any complication related to treatment.
Radiotherapy is an effective treatment for CD20 negative PTLD and must be considered as a treatment option in this kind of PTLD subtype mostly unresponsive to Rituximab.
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