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Editor,—Human T cell lymphotropic virus type I (HTLV-I) has been shown to cause two systemic diseases: a haematological malignancy termed adult T cell leukaemia/lymphoma (ATL)1 and a chronic progressive neurological disease, known as HTLV-I associated myelopathy or tropical spastic paraparesis (HAM/TSP).23 Among HTLV-I infected individuals, a certain type of uveitis has been reported,4 which responds to corticosteroids and visual outcome is usually favourable.5 Although perinatal virus transmission occurs frequently via breast feeding and saliva,6 the HTLV-I seroprevalence increases with age.4 We report here a child with severe HTLV-I associated uveitis (HAU).
CASE REPORT
A 12-year-old girl was admitted with abrupt onset of blurred vision and decreased visual acuity in the right eye. Her visual acuity was right eye, 0.02 and left eye, 0.8. Ocular examination revealed vitreous and retinal haemorrhages in the right eye and scarred retinal vasculitis in the left eye. She was suspected to have an acute posterior and intermediate uveitis. Physical examination demonstrated no evidence of arthritis, pulmonary involvement, hepatosplenomegaly, or neurological abnormalities. Laboratory examinations of peripheral blood, coagulation studies, autoantibodies to nuclear antigen and rheumatoid factor revealed no systemic abnormalities, and studies of antibodies to viruses including rubella, cytomegalovirus, herpes simplex virus, Epstein–Barr virus, varicella zoster virus, and Toxoplasma gondii indicated previous infections or no evidence of infection. Examination of the patient’s serum revealed the antibody to HTLV-I by both particle agglutination assay (PA) and ELISA, which was confirmed by western blot. The PA titre for HTLV-I was initially 1:256 and increased to 1:8192 over the next 4 months. Provirus detection by polymerase chain reaction (PCR) using peripheral blood mononuclear cells was positive for HTLV-I DNA. The patient’s mother was an asymptomatic carrier of HTLV-I. The girl was, therefore, diagnosed as having HTLV-I associated uveitis (HAU). Along with topical steroid eyedrops, 1 ml of intravenous liposteroid, consisting of lipid emulsified dexamethasone (L-Dx) microspheres (Limethason 1 ml; dexamethasone palmitate 4 mg)7 was injected twice a week in order to target steroids to the topical, ocular inflammatory lesion and to minimise adverse effects.7 By 3 weeks, the uveitis had nearly resolved, and injections of L-Dx were decreased. Her visual acuity of the right eye improved to right eye, 0.3. Six weeks later, a flaring of uveitis and retinal bleeding with iritis in right eye after a flu-like infection occurred. Visual acuity was right eye, 0.01 and left eye,1.0. L-Dx injections were initiated twice a week again, and the acute inflammation improved within 3 weeks. However, retinal detachment and vitreous opacities in the right eye ultimately developed, which prompted surgical treatment (pars plana vitrectomy, three port system, 20% SF6 exchange) (Figs 1 and 2). The fluid in the anterior chamber contained antibodies to HTLV-1 (1:160 by PA assay).
Fundus photograph of the right eye 3 weeks after vitrectomy. Diffuse vitreous opacities, irregular dilatation of retinal vessels, and subretinal exudate. Segmental protrusion is seen at the inferotemporal quadrant after buckling surgery for an iatrogenic break.
Fundus photograph of the left eye. Membranous tent-like vitreous opacity is seen in the periphery.
COMMENT
We report here a child with severe HAU associated with carriage of HTLV-I. The age of onset of HAU has been reported to be distributed from 19 years to 75 years (mean 46.03 (SD3.1) years),8making our patient one of the youngest reported to date.
The diagnosis of HAU is based on the sudden onset of uveitis without defined causes, in addition to a seropositive result for antibodies to HTLV-I.9 Although some patients with ATL or HAM/TSP develop idiopathic granulomatous anterior uveitis, patients with HAU show no signs of neurological or haematological abnormalities except for antibodies to HTLV-I.9 Our patient satisfied these two criteria, and the titre of anti-HTLV-I antibody increased during the course of her disease. Moreover, proviral DNA was positive, which further contributed to confirm the diagnosis.
Unilateral intermediate and posterior uveitis was the initial ocular lesion in this case, ultimately progressing to panuveitis in both eyes. This explains the poor ocular prognosis in this child, who needed surgical therapy, although the visual acuity of most HAU patients is good (63%) or fairly good (24%).10 The retinal detachment presumably occurred by the traction of vitreous with neovascularisation as we could not find any retinal break during the vitreous surgery. Although this patient was free from haematological and neurological manifestations, it remains to be established whether HAU is predictive of the subsequent development of other HTLV-I associated diseases.