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We read with interest the report of Kalpoe et al.1 concerning varicella zoster-associated progressive outer retinal necrosis (PORN) after allogeneic stem cell transplantation, and describe another, similar case.
This journal is a member of and subscribes to the principles of the Committee on Publication Ethics. The patient is currently alive and well, although with profound visual impairment.A 51-year-old man was diagnosed with refractory anaemia with excess blasts type one (RAEB 1) and treated with two cycles of FLAG-Ida chemotherapy.
Varicella Zoster virus (VZV) related progressive outer retinal necrosis (PORN) after allogeneic stem cell transplantation. He then underwent a reduced intensity conditioned matched unrelated donor stem cell transplant using fludarabine, melphalan and Campath I-H as conditioning.
He had a good response and was recommenced on a prophylactic dose of 200 mg acyclovir three times a day in view of his on-going lymphopenia and immunosuppression.

During this period, plasma samples for qualitative VZV PCR were strongly positive, although levels were not quantitated.At 7 months post transplant, while still receiving cyclosporine, he became increasingly dyspnoeic.
Bilateral reticulonodular shadows were present on chest X-ray, and a CT scan showed bronchiectasis and peripheral fibrosis.
Bronchoscopy and lavage revealed no abnormalities and a clinical diagnosis of lung graft-versus-host disease was made. Mycophenolate mofetil was commenced in addition to cyclosporin when the steroid dose was reduced, in view of the diagnosis of probable lung graft-versus-host disease and the fact that he was still suffering from significant shortness of breath.After 1 month, he presented with a 5-day history of a left visual field defect. The left fundus showed extensive peripheral pigmented mottling with a small, isolated haemorrhage while the right showed more localised and less marked change. A CT scan of the brain excluded haemorrhage, and MRI scans of the brain and orbits were normal.He continued on oral valaciclovir, and was referred to a specialised retinal unit where the possibility of additional old inflammatory changes was considered and a differential diagnosis of cytomegalovirus (CMV) was suggested.

At this stage a diagnosis of bilateral progressive outer retinal necrosis (PORN) was made and VZV infection was confirmed by PCR on vitreal biopsy material. He was subsequently discharged taking oral valacyclovir 1 g three times daily and continues under regular review.
Plasma PCR for VZV now showed only weak positivity.He describes progressive dimming of his vision which he likens to the illumination provided by light bulbs of increasingly low Wattage.
He can currently see only large shapes in shades of grey with no colour, but feels that his vision has now stabilised.This case further highlights an unusual complication of stem cell transplantation, which needs aggressive investigation and combination therapy in order to maximise the chances of conserving as much vision as possible.

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