Immune recovery disease: a case of interstitial keratitis and tonic pupil following bone marrow transplantation
- A S Ioannidis1,
- M Forrest1,2,
- K K Nischal1,2,
- P Veys3,
- E G Davies3,
- G Woodruff4
- 1Department of Ophthalmology, Great Ormond Street Hospital for Children, London, UK
- 2Department of Visual Sciences, Institute of Child Health, London, UK
- 3Department of Bone Marrow Transplantation and Immunology, Great Ormond Street Hospital for Children, London, UK
- 4Department of Ophthalmology, Leicester Royal Infirmary, Leicester, UK
- Correspondence to: Mr K K Nischal Department of Ophthalmology, Great Ormond Street Hospital, Great Ormond Street, London WC1 3JH, UK; kknbtinternet.com
- Accepted 26 April 2004
Immune recovery disease results from an immunological response to circulating viral antigens in the host after bone marrow transplant (BMT) mediated immune reconstitution. It may also occur after successful antiretroviral therapy in patients with HIV and AIDS. We report a case of a child with severe combined immune deficiency (SCID) and disseminated varicella zoster virus (VZV) infection who developed interstitial keratitis and a tonic pupil after BMT.
CASE REPORT
An 8 month old male infant was referred to the ophthalmology clinic at Great Ormond Street because of suspected congenital glaucoma. The past ophthalmic and family history were unremarkable. The child was born with multiple congenital anomalies of the lower limbs which included bilateral tibial deficiencies, and an extreme talipes equinovarus of the right foot.
The child had a known history of disseminated varicella infection caused by SCID (fig 1). On examination it was noted that he had a generalised vesicular rash throughout his body extending to his eyelid margins. The eyes were white with clear corneas and he was alert, fixing and following well with full extraocular eye movements. Both pupils were reactive to light with no afferent pupillary defect. The anterior chambers were unremarkable and the intraocular pressure with the Perkins tonometer was 16 mm Hg bilaterally. Examination of the fundus, including cup to disc ratio was normal. He was reviewed periodically over the next 6 months while an inpatient undergoing treatment for SCID. During this period, he was persistently positive for VZV DNA in his blood determined by polymerase chain reaction (PCR) analysis but was negative for Epstein-Barr virus (EBV), herpes simplex virus (HSV), cytomegalovirus (CMV), and adenovirus DNA. He suffered a …







