Cytomegalovirus retinitis in the absence of HIV
or immunosuppression
D.J. SLOAN
1,2
, M. TAEGTMEYER
1,2
, I.A. PEARCE
3
, I.J. HART
4
, A.R.O. MILLER
1,2
, N.J. BEECHING
1,2
1
Tropical and Infectious Disease Unit, Royal Liverpool University Hospital
2
Liverpool School of Tropical Medicine
3
St Pauls Eye Unit, Royal Liverpool University Hospital
4
Department of Virology, Royal Liverpool University Hospital, Liverpool - UK
INTRODUCTION
Cytomegalovirus (CMV) is associated with sight-threaten-
ing retinitis in human immunodeficiency virus (HIV) infec-
tion at CD4+ counts below 50 cells/μL. Systemic steroids
are an independent risk factor for the development of
clinical disease in this group (1). Less commonly, non-HIV
related CMV retinitis occurs in transplant recipients or
heavily immunosuppressed patients with autoimmune
European Journal of Ophthalmology / Vol. 18 no. 5, 2008 / pp. 813-815
1120-6721/813-03$25.00/0 © Wichtig Editore, 2008
PURPOSE. Cytomegalovirus (CMV) retinitis classically occurs in advanced human immunod-
eficiency virus (HIV) infection but is rare in other forms of immunosuppression. The authors
report a case of CMV retinitis in an HIV-negative man with idiopathic CD4 lymphocytope-
nia (ICL). This is the first such case to be confirmed by polymerase chain reaction (PCR) of
aqueous humor.
METHODS. Case report.
RESULTS. A 69-year-old retired Chinese seaman presented with gradual visual deterioration.
He was a diet controlled diabetic on regular steroids for presumed asthma. Examination
showed no diabetic eye disease but confirmed acute retinal necrosis (ARN). Anterior cham-
ber tapping of the aqueous humor was PCR positive for CMV. HIV antibody and RNA tests
were negative but his full blood count revealed lymphocytopenia, with a low CD4+ subset.
He responded to a 3-week course of intravenous ganciclovir therapy followed by suppres-
sive oral valganciclovir.
CONCLUSIONS. CMV is associated with sight-threatening retinitis in HIV infection at CD4+
counts below 50 cells/μL and in transplant recipients or heavily immunosuppressed pa-
tients. Systemic steroids are a risk factor for clinical disease in these groups. It is extremely
rare to report CMV eye disease in previously healthy individuals. This case illustrates that
the condition does occur in association with ICL. Corticosteroids may be implicated in dis-
ease reactivation. Molecular methods are necessary to confirm the diagnosis. (Eur J Oph-
thalmol 2008; 18: 813-5)
KEY WORDS. Cytomegalovirus, Retinitis, HIV negative, CD4 lymphocytopenia, Corticosteroids
Accepted: March 16, 2008
SHORT COMMUNICATIONS & CASE REPORTS
disease (2). It is extremely rare to report CMV eye disease
in previously healthy individuals and corticosteroids have
been implicated as the sole immunomodulatory factor on
only three occasions (3-5).
We present a case of acute retinal necrosis (ARN) sec-
ondary to confirmed CMV infection in an HIV-negative man
on low dose steroids for asthma with a presenting CD4+
count of 65 cells/μL. The explanation for his lymphocytope-
nia and issues concerning ongoing therapy are discussed.