Dados do Trabalho

Título

A rare case of primary ocular Post-Transplant Lymphoproliferative Disorder in a Brazilian child.

Resumo

Objectives: To describe ocular disease in a patient with post-transplant lymphoproliferative disorder (PTLD) and to identify the frequency of such ocular involvement.

Methods: The case history is described of an eight-year-old boy that underwent a single kidney transplant for congenital bilateral renal hypoplasia and was subsequently on immunosuppressive treatment with tacrolimus and prednisone. After four years of transplantation, he presented with two “lumps on the iris”, without visual disturbance or ocular symptoms. An incisional biopsy of an iris nodule in the left eye was performed and light microscopic examination showed a dense collection of plasma cells and slightly atypical lymphocytes. Immunoperoxidase staining showed an equal mixture of λ and κ light chains. In situ hybridization studies for Epstein-Barr virus (EBV) were positive. A diagnosis of PTLD was made based on these results.

Discussion: PTLD represents a spectrum of diseases associated with EBV infection in up to 80% of cases in the setting of pharmacologic immunosuppression subsequent solid organ transplantation. Ocular involvement is a rare finding in PTLD, we identified only four cases of intraocular involvement in the medical literature. Brodsky et al reported the case of a 7-year-old girl who was submitted to liver transplantation and was immunosuppressed with cyclosporine. She developed bilateral iris. Robinson et al reported the case of a 2-year-old girl who was receiving cyclosporine after liver transplantation and developed multiple pigmented iris nodules. Clark et al reported a case of intraocular PTLD in a 2-year-old boy who developed unilateral iris tumor. This patient was immunosuppressed with cyclosporine and prednisone after undergoing liver transplantation. Demols et al reported the case of a 59-year-old man with PTLD after lung transplantation, who developed a unilateral iris nodule.

Conclusion: This case shows a rare presentation of ocular PTLD with iris involvement and highlights on the diagnostic of this entity.

Área

Leucemia e/ou linfoma