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

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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.

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Acknowledgements

We would like to express our thanks to PD Dr. M. Hummel, Paulinenkrankenhaus Berlin, Germany, for considerable diagnostic and therapeutic suggestions. Prof. Dr. H.-D. Volk and Prof. Dr. P. Reinke, Charité Hospital Berlin, have kindly suggested and organized the immunologic therapy. Finally, we thank Dr. A. Reuscher, Esslingen, Germany, for referral of the patient, and the parents of the child for giving the permission for publication.

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Authors and Affiliations

  1. Department 1, University Eye Clinic, Schleichstr. 12, 72076, Tübingen, Germany

    Jens Martin Rohrbach & Manfred Zierhut

  2. Institute for Pathology, University of Tübingen, Tübingen, Germany

    Stefan Martin Kröber

  3. Pediatric Cardiology, Stuttgart, Germany

    Thomas Teufel

  4. University Clinic for Radiooncology, University of Tübingen, Tübingen, Germany

    Rolf-Dieter Kortmann

Authors
  1. Jens Martin Rohrbach
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  2. Stefan Martin Kröber
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  3. Thomas Teufel
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  4. Rolf-Dieter Kortmann
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  5. Manfred Zierhut
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Corresponding author

Correspondence to Jens Martin Rohrbach.

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Rohrbach, J.M., Kröber, S.M., Teufel, T. et al. EBV-induced polymorphic lymphoproliferative disorder of the iris after heart transplantation. Graefe's Arch Clin Exp Ophthalmol 242, 44–50 (2004). https://doi.org/10.1007/s00417-003-0751-4

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  • DOI: https://doi.org/10.1007/s00417-003-0751-4

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