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Varicella zoster virus (VZV)-related progressive outer retinal necrosis (PORN) after allogeneic stem cell transplantation

Bone Marrow Transplantation volume 36, pages 467–469 (2005)Cite this article

Commonly reported complications of disseminated varicella zoster virus (VZV) infections in severely immunocompromised patients (eg stem cell transplant recipients) include VZV pneumonia, encephalitis and hepatitis. Progressive outer retinal necrosis (PORN) is described as a distinct form of VZV necrotizing chorioretinitis and is found almost exclusively in patients with acquired immunodeficiency syndrome (AIDS).1, 2, 3, 4 However, a few cases in non-AIDS patients have been reported. These patients were immunocompromised due to therapy for idiopathic thrombocytopenic purpura (ITP), cutaneous non-Hodgkin's lymphoma, rheumatoid arthritis and renal transplantation.1 To our knowledge no case of PORN, with laboratory confirmation of VZV involvement, occurring after allogeneic stem cell transplantation (allo-SCT) has been reported until now. Here, we report a laboratory confirmed case of PORN following disseminated VZV infection in an allo-SCT patient.

After 4 weeks of the start of symptoms in the left eye the patient complained of decreased vision with the right eye and ophthalmic examination of the right eye revealed a visual acuity of 1.0 and an unremarkable anterior segment without vitreous cells. Fundus examination showed coalescing peripheral retinal lesions with temporal opacifying retinal necrosis (Figure 2b). Again the presence of VZV DNA was demonstrated in right anterior segment fluid and HSV, CMV and EBV DNA were undetectable. Aciclovir was replaced by foscarnet: intravenous (24 mg/ml t.i.d.) and intravitreal in the right eye (1.2 mg/0.05 ml three times a week). Laser coagulation was performed to separate necrotic from healthy retina. After 1 week, signs of recovery (retinal traction) were seen and surgery as carried out for the left eye, was performed. After the operation visual acuity of the right eye dropped to 0.4 but no progression was seen with regard to the retinitis. At 6 months after transplantation, T-cell lymphoma relapsed and after 4 weeks the patient died of progressive lymphoma.

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Authors and Affiliations

  1. Department of Medical Microbiology, E4-69, Leiden University Medical Center, PO Box 9600, RC Leiden, 2300, The Netherlands

    J S Kalpoe, N Vaessen, E C J Claas, A C M Kroes & M F C Beersma

  2. Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands

    C E van Dehn & J G Bollemeijer

  3. Department of Hematology, Leiden University Medical Center, Leiden, The Netherlands

    R M Barge & R Willemze

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  1. J S Kalpoe
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  2. C E van Dehn
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Kalpoe, J., van Dehn, C., Bollemeijer, J. et al. Varicella zoster virus (VZV)-related progressive outer retinal necrosis (PORN) after allogeneic stem cell transplantation. Bone Marrow Transplant 36, 467–469 (2005). https://doi.org/10.1038/sj.bmt.1705085

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