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Primary intraocular posttransplantation lymphoproliferative disorder

W L Clark1, I U Scott, T G Murray

  • 1Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Fla., USA.

Archives of Ophthalmology (Chicago, Ill. : 1960)
|December 31, 1998
PubMed
Summary

We present a rare case of posttransplantation lymphoproliferative disorder (PTLD) in a young child, presenting as an isolated eye tumor. This finding highlights PTLD

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Area of Science:

  • Pediatric Oncology
  • Transplantation Immunology
  • Ophthalmic Pathology

Background:

  • Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication following organ transplantation.
  • PTLD typically involves lymphoid proliferation, often associated with Epstein-Barr virus (EBV) reactivation.
  • Ocular manifestations of PTLD are uncommon, with isolated iris involvement being exceedingly rare.

Observation:

  • A 2-year-old boy, who received a liver transplant for biliary atresia at 4 months old, presented with a progressively enlarging iris nodule over two months.
  • The patient had no other signs or symptoms suggestive of systemic PTLD at the time of presentation.
  • Clinical examination revealed a unilateral, isolated iris mass.

Findings:

  • Histopathologic examination of the iris lesion confirmed a diagnosis of PTLD.

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  • The tumor comprised a mixed population of lymphoid cells, consistent with PTLD.
  • This case represents the youngest reported patient with PTLD manifesting solely within the eye.
  • Implications:

    • This case underscores the importance of considering PTLD in the differential diagnosis of pediatric ocular tumors, especially in transplant recipients.
    • Early recognition and diagnosis of ocular PTLD are crucial for timely management and potentially preventing systemic dissemination.
    • Further research into the specific mechanisms and risk factors for isolated ocular PTLD in pediatric transplant patients is warranted.