A man in his 60’s had a history of chronic lymphocytic leukaemia (CLL) treated with rituximab plus bendamustine (r-bendamustine) five years previously, with complete remission. 

He presented with left-sided herpes zoster ophthalmicus (and was treated with oral aciclovir. At further review, he was noted to have right-sided acute retinal necrosis. Empirical intravitreal foscarnet was given, and oral high dose valaciclovir was commenced. Anterior chamber tap confirmed varicella zoster virus.

While still taking high-dose oral valaciclovir, he developed right-sided limb weakness and facial asymmetry. CT and MR imaging identified left middle carotid artery infarction.

His varicella vasculopathy was managed first with iv foscarnet, then oral valaciclovir. 

4 months after his initial presentation he developed sudden vision loss in the right eye due to macular-on rhegmatogenous retinal detachment. He underwent successful primary repair with 360-degree laser and silicon oil fill. After removal of oil, his vision is preserved at 0.44 logMAR.

The reactivated varicella virus travels from the trigeminal ganglion along trigeminal axons to reach the cerebral arteries. The virus migrates into vessel walls, provoking an immune reaction and amyloid production. This results in an occlusive cerebral vasculitis which can lead to complications such as ischaemia, infarction or haemorrhage.

The mainstay of acute management is intravenous antiviral treatment for at least 2 weeks e.g. Aciclovir 15mg/kg TDS or Foscarnet 90mg/kg BD. There is an argument for the role of steroid in reducing vascular inflammation e.g. 1 mg/kg from day 1-5 of the antiviral course. We recommend discussion with Regional Virology services for specific guidance.

Patients with CLL treated with r-bendamustine have lifelong immune suppression which persists even when the disease is in remission. There is no international consensus on the optimal duration of herpesvirus prophylaxis , however long-term prophylaxis extending years is recommended to mitigate the considerable morbidity associated with herpesvirus reactivation in this population.