Statistics from Altmetric.com
Editor,—Ocular involvement has commonly been reported in patients with acute leukaemias.1-3 Although acute lymphoblastic leukaemia (ALL) may present as uveitis, this presentation has rarely been reported in patients with acute myeloid leukaemia (AML).4 We describe an adult male who initially presented with an anterior uveitis followed by the rapid development of orbital involvement who was subsequently diagnosed with AML.
A 43 year old man presented to the emergency room with a 5 day history of photophobia and redness of the right eye with a precipitous decrease in vision over the previous 24 hours. He had been seen 2 days earlier by another ophthalmologist and was found to have retinal vasculitis and anterior uveitis. He was started on oral corticosteroids and referred to an internist for systemic examination for the presumptive diagnosis of Behçet’s disease. His medical history was remarkable for anal and mouth abscesses, 2 months before his visit.
On examination visual acuity was 6/60 right eye and 6/6 left eye. Ocular examination of the left eye was unremarkable. There was conjunctival injection, keratic precipitates, +2 anterior chamber cells, 360 degrees of iris synechiae, and an inflammatory membrane covering the anterior surface of the lens. The anterior chamber was deep and the intraocular pressure was within the normal range. Examination of the right eye revealed no eyelid swelling or proptosis. Funduscopic examination of the right eye was not possible because of the pupillary membrane. A diagnosis of uveitis was made, an examination was begun including HLA B27 and HLA B51 and the patient was started on frequent topical corticosteroids.
Three days later the patient developed an acute onset of severe eyelid swelling, pain, and proptosis of the right eye (Fig 1). Visual acuity of the right eye was counting fingers and the intraocular pressure was elevated at 44 mm Hg. A complete blood cell count (CBC) and peripheral blood smear revealed predominance of blast cells, and a diagnosis of AML was made. B-scan ultrasonography of the right eye revealed vitreous opacities, a tractional retinal detachment temporally, thickened ocular walls, and a mass lesion in the posterior orbit. An emergent computed tomograph scan confirmed the presence of an infiltrating orbital mass (Fig 2) and an urgent canthotomy, cantholysis, and orbital biopsy were performed. The biopsy demonstrated evidence of focal aggregates of mononuclear cells with cleaved nuclei consistent with leukaemic infiltrate. With a presumptive diagnosis of orbital leukaemic infiltrate, the patient underwent orbital irradiation in conjunction with systemic broad spectrum antibiotics. He responded rapidly with a decrease of lid swelling, proptosis, and the intraocular pressure within 24 hours. Immunohistochemical staining of the orbital biopsy was not able to demonstrate conclusively the presence of leukaemic cells.
The patient subsequently underwent an unsuccessful bone marrow transplant, and died 3 months after the initial presentation. Postmortem examination of the orbital biopsy was not performed.
Although uveitis is commonly reported in children with relapsing acute leukaemias, it rarely is the first presentation of AML.4 5 Leukaemic retinopathy, including haemorrhages, cotton wool spots, and retinovascular abnormalities are the most common ocular manifestations in patients with AML.1-4 Anterior segment and vitreous findings are rarely described in these patients. In a prospective study of 56 patients with AML, 53% of the patients had ocular manifestations at the time of diagnosis, but none had anterior segment or vitreous involvement.6
In our patient, the initial presentation with anterior iridocyclitis did not raise suspicion of malignancy. However, the recent medical history of anal and mouth abscesses in a previously healthy young man was suggestive of an immunocompromised host. The worsening of anterior chamber inflammation despite aggressive topical application of steroid was followed in several days by signs of rapid orbital involvement. Posterior segment involvement was not seen at the time of presentation because of a dense pupillary membrane, but was later documented on B-scan echography. The diagnosis of retinal vasculitis by the previous ophthalmologist most likely represented leukaemic retinopathy secondary to perivascular infiltration by the leukaemic cells. The orbital involvement in this patient may have been due to leukaemic infiltration, orbital haemorrhage, or orbital cellulitis. Although the diagnosis of leukaemic infiltrate was not immunohistochemically confirmed, the rapid response to irradiation, and the pattern of intraocular as well as retrobulbar involvement pointed towards this diagnosis.
The delay in the diagnosis of acute myeloid leukaemia in our patient was minimised by the rapid progression of the disease, quickly leading to further investigations. However, the question arises whether an initial CBC should be performed on all patients with anterior iridocyclitis. In our institution, of 534 adult patients treated for uveitis over the past 3 years, five cases were secondary to intraocular tumours, and only the present case was associated with an acute leukaemia. This low incidence argues against the use of a CBC for screening of previously healthy adults with typical anterior uveitis. However, in cases of worsening inflammation despite frequent topical steroids a CBC with smear may be recommended.
Presented at the annual meeting of the Canadian Ophthalmological Society on 28 June 1998 in Calgary, Alberta.