Diagnosis by polymerase chain reaction of ocular posttransplant lymphoproliferative disorder after pediatric renal transplantation.

PURPOSE: To report diagnosis by polymerase chain reaction of intraocular posttransplant lymphoproliferative disorder in a pediatric renal transplant patient. DESIGN: Observational case report. METHODS: Retrospective review. RESULTS: An 11-year-old girl developed infectious mononucleosis 15 months after renal transplantation for focal segmental sclerosis. Papillitis and hypopyon uveitis developed 8 months later, followed by iris nodules. Diagnosis of intraocular posttransplant lymphoproliferative disorder was made by polymerase chain reaction of aqueous humor for Epstein-Barr virus and confirmed by histopathology of an iris biopsy specimen. Infiltrating iris lymphocytes in the biopsy specimen were positive for Epstein-Barr DNA. Polymerase chain reaction also revealed gene rearrangement of the variable region of the heavy immunoglobulin chain, consistent with a monoclonal B-lymphocyte population. Iris nodules resolved with reduction in immunosuppressive medication. CONCLUSION: Polymerase chain reaction for Epstein-Barr virus may be helpful in diagnosis of intraocular posttransplant lymphoproliferative disorder.

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

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.

Herpes zoster sine herpete. A potential cause of iridoplegic granulomatous iridocyclitis.

OBJECTIVE: Herpes zoster ophthalmicus (HZO) is a recurrence of varicella zoster virus involving cranial nerve V-1, but does not always have skin manifestations. The objective of this work is to study iridoplegic granulomatous iridocyclitis as an acute, fulminant iridocyclitis that probably is caused by the recurrence of varicella zoster virus without skin eruptions. PARTICIPANTS: The author reports 15 cases of iridoplegia granulomatous iridocyclitis with involvement of the anterior uveal tract without known skin eruptions. RESULTS: All patients have had a clinical course of iridocyclitis closely resembling those cases of herpes zoster with skin eruptions. Nine of the 15 are documented to have had a recurrence of varicella zoster virus with an appropriate rise and fall of systemic titers. The remaining six patients had clinical findings, including loss of accommodation, iridoplegia, and sectoral iris atrophy that were more typical for HZO than other infectious agents. CONCLUSIONS: Iridoplegic granulomatous iridocyclitis is a newly described, acute, fulminant uveitis probably caused by a herpes virus and most probably by varicella zoster virus. Herpes zoster sine herpete (erupticum) should be suspected as a potential diagnosis in patients with appropriate anterior segment manifestations. Further study is necessary to discern if any of such cases could be caused by herpes simplex.

Detection of herpes simplex viral DNA in the iridocorneal endothelial syndrome.

OBJECTIVE: To test the hypothesis that the iridocorneal endothelial (ICE) syndrome has a viral origin by comparing the incidence of viral DNA in corneal specimens from patients with the ICE syndrome and from controls. DESIGN: Thirty-one corneas obtained from 25 patients with the ICE syndrome and six with chronic herpetic keratitis (n = 31) were compared with 30 control specimens obtained from 15 healthy donors and from 15 patients with other, nonviral chronic corneal diseases. METHODS: Primer pairs and polymerase chain reaction methods were used to identify and amplify either a segment of the DNA polymerase gene in the case of the herpes simplex and zoster viruses or a region of the nuclear antigen gene for the Epstein-Barr virus. The oligonucleotide amplified by polymerase chain reaction was fully characterized with the use of restriction enzyme, hybridization, and sequence analyses to determine that it contained the expected base pair sequence. RESULTS: Sixteen of 25 ICE syndrome specimens and four of six herpetic keratitis specimens were positive for herpes simplex virus (HSV) DNA. All nine ICE syndrome specimens tested were negative for the presence of DNA from the herpes zoster or the Epstein-Barr viruses. Controls were uniformly negative for HSV DNA whether they were obtained from ostensibly normal corneas (n = 15) or from corneas with intestinal keratitis, aphakic bullous keratopathy, or keratoconus (n = 15). Tissue samples cut from positive ICE syndrome specimens yielded negative results when retested after the endothelial layer was removed. These findings indicate that localization of HSV DNA is within the endothelium, the tissue primarily involved in the pathogenesis of the ICE syndrome. CONCLUSIONS: Polymerase chain reaction evidence shows that HSV DNA is present in a substantial percentage of ICE syndrome corneal specimens and that HSV-DNA is absent in normal corneas and in corneas from patients with three other chronic corneal diseases. These results provide direct evidence to support our hypothesis that the ICE syndrome has a viral origin. We discussed clinical implications, including possible therapeutic interventions.